Paediatric Nephrology Flashcards

1
Q

What are the five main functions of the kidney?

A
  1. Waste handling
  2. Water handling
  3. Salt balance
  4. Acid base control
  5. Endocrine (erythropoeitin / RAAS / PTH)
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2
Q

What are two of the main presenting symptoms in nephrology?

A

Haematuria (indicative of nephritic syndrome)

Proteinuria (indicative of nephrotic syndrome)

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3
Q

What is proteinuria a sign of?

A

Glomerular injury

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4
Q

Which glomerulopathies tend to affect the epithelial cells (podocytes) of the glomerulus? The basement membrane? The Endothelial cells? The mesangial cells?

A

Podocyte - minimal change disease

Basement membrane - post infective glomerulonephritis (PIGN)

Endothelial cells - PIGN, haemolytic uraemic syndrome (HUS)

Mesangial cells - HSP / IgA nephropathy

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5
Q

What are the different methods of detecting and quantifying protein in the urine?

A

Dip stick - (>3+ abnormal) measures concentration

Protein : Creatinine ratio - early morning urine is best (normal < 20, nephrotic >250)

24hr urine collection (gold standard, not that practical)

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6
Q

Descrube the pathophysiology of nephrotic syndrome?

A

Nephrotic syndrome is characterized by injury to the glomeruli which leads to excessive proteinuria

This causes hypoalbuminaemia

This leads to significant oedema

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7
Q

How does nephrotic syndrome tend to present?

A

Significant oedema (pitting, periorbital, ascites, small pleural effusions)

Can be hyper, hypo or normo tensive

Frothy urine

Inflated weight

Microscopic haematuria & low urine Na also common

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8
Q

Investigations for suspected nephrotic syndrome?

A

Urine protein, urine Na

Protein creatinine ratio (urine)

Bloods: albumin, creatinine

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9
Q

What is the most common kidney pathology in children? What clinical features would be expected?

A

Minimal change disease

  • Normal blood pressure
  • No frank haematuria
  • normal renal function
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10
Q

How is minimal change disease treated in children? What are some possible side effects of treatment?

A

Prednisolone for 8 weeks (anti-inflammatory)

Personality change
Susceptibility to infection
Hypertension
Increased acid production (GI)

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11
Q

What is one characteristic of MCD with regards to treatment that helps separate it from other glomerulonephropathies?

A

MCD tends to respond to steroid treatment

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12
Q

What is the prognosis for MCD in children like?

A

It’s good

80% experience long term remission, others require long time immunosuppression

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13
Q

What causes MCD?

A

Lympocytes (Ts and Bs) attack podocytes of the glomerulus

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14
Q

What is the most common acquired cause of steroid resistant nephrotic syndrome?

A

Focal segmental glomerulosclerosis

Characterized by podocyte loss and progressive inflammation and sclerosis

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15
Q

What is the course of action if haematuria is detected in a child?

A

Frank haematuria - always investigate

Microscopic haematuria - positive 3 times and then investigate, or positive once with accompanying proteinuria

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16
Q

What are some possible causes of haematuria?

A
Glomerulonephritis 
Malignancies - sarcomas 
UTI 
Stones 
Trauma 
Urethritis
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17
Q

What component of the glomerulus is usually injured in the presence of frank haematuria?

A

Endothelial cell

  • Post infective glomerulonephritis
  • Haemolytic uraemic syndrome
  • Membranoproliferative Glomerulonephritis
  • Lupus
  • ANCA vasculitis
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18
Q

What investigations may be done to check for post infective glomerulonephritis?

A

ASOT (positive - checks for strep A)

Bacterial culture

Urine dipstick (blood / protein)

Renal USS

Immunology workup (C3 & C4)

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19
Q

What are some symptoms that may be seen in acute post infective glomerulonephritis?

A

Haematuria (+/- proteinuria)

Hypertension

Oedema

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20
Q

What is the disease course of post infective glomerulonephritis usually like? How is it treated?

A

Usually self limiting

Treat with antibiotics and support of renal function:

  • Electrolytes / acid base
  • Diuretic if hypertensive
21
Q

What is the most common glomerulonephritis? When does it tend to occur? Who does it tend to occur in?

A

IgA nephropathy

Tends to occur shortly following an URTI

Usually occurs in older children and adults

22
Q

How does IgA nephropathy tend to present?

A

Recurrent frank haematuria +/- chronic microscopic haematuria

Varying degrees of proteinuria

23
Q

What is the gold standard for diagnosis of IgA nephropathy?

A

Kidney biopsy

  • Should be IgA deposits in the mesangial cells
24
Q

How is IgA nepropathy treated?

A

Mild disease: ACEi for hypertension and proteinuria

Moderate to severe disease: immunosuppression

25
Q

What is the cardinal sign of IgA associated vasculitis?

A

Palpable purpura (blood spots on the skin)

  • IgA collects in the blood vessels and causes inflammation and leaking of blood
26
Q

In addition to purpura, what other symptoms are needed for diagnosis of IgA associated vasculitis?

A

One of the following 4:

  • Abdominal pain
  • Renal involvement
  • Arthritis or arthralgia
  • Skin Biopsy: IgA deposition
27
Q

What causes IgA vasculitis? What is the disease course like?

A

Usually occurs 1-3 days post trigger (viral URTI, streptococcus, drugs)

Duration of symptoms is usually 4-6 weeks (1/3 of patients relapse)

can result in mesangial cell injury

28
Q

How is IgA vasculitis treated?

A

Glucocorticoid therapy

Immunosuppression

Hypertension and proteinuria screening

29
Q

What are some of the cardinal signs of acute kidney injury (AKI)?

A
  • Anuria / oliguria (<0.5mL/kg/hr) for 8 hours
  • Hypertension with fluid overload
  • Rapid rise in plasma creatinine (>1.5x normal level for the childs age)
30
Q

What causes pre-renal AKI?

A

Any condition leading to hypoperfusion of the glomerulus

gastroenteritis / acute haemorrhage

31
Q

What are some causes of intrinsic renal AKI?

A

Glomerular disease - haemolytic uraemic syndrome, glomerulonephritis

Tubular injury - acute tubular necrosis (as a consequence of hypoperfusion / drugs)

Interstitial nephritis - NSAIDs, autoimmune

32
Q

What are some causes of post-renal AKI?

A

Obstructive uropathies

conditions leading to the obstruction of the ureters

33
Q

What are some of the characteristic findings in haemolytic uraemic syndrome?

A

Haemolysis (packed cell volume (PCV) less than 30% / Hb level <10)

Thrombocytopenia (platelet count >150)

Raised serum creatinine (>97th percentile)

GFR decreased

Proteinuria

34
Q

What causes haemolytic uraemic syndrome (HUS)?

A

Typical HUS:

  • Occurs post diarrhoea
  • Other causes (pneumococcal infections / drugs)

Atypical HUS:
- genetic condition due to overactive / inappropriate activation of the complement system

35
Q

What organism tends to cause the bloody diarrhoea that may lead to HUS in children?

A

E. Coli

  • Bloody diarrhoea is a medical emergency in children
36
Q

What conditions is HUS a syndrome of?

A

Microangiopathic haemolytic anaemia

Thrombocytopenia

AKI / acute renal failure

37
Q

What is the best management technique for haemolytic uraemic syndrome?

A

Intravascular volume expansion

  • Monitor kidney function
  • Maintain fluid volume: IV saline and fluid**, renal replacement therapy
  • Avoid antibiotics / NSAIDS
38
Q

What is the general rule for managing acute kidney injuries?

A

3M’s:

  • Monitor: 5 kidney functions (fluid balance, electrolytes, acid base, endocrine, waste)
  • Maintain: fluid output (IV saline and renal replacement therapy)
  • Minimise drugs
39
Q

How far does renal function have to drop before creatinine becomes abnormal? How does this look in terms of eGRF?

A

Renal function has to drop around 40%

Normal GFR - 120
Creatinine abnormal at around GFR - 60
Kidney failure at around GFR - 15

40
Q

How do children with chronic kidney disease tend to present?

A

Increased urea (loss of apetite & itchiness)

Polyuria (or oliguria…)

Salt balance (hyponatraemia - abnormal growth, hypokalaemia - cardiac symptoms)

Acid base balance (can also affect growth)

Endcrine (anaemic, hypotensive, bad bone health due to PTH)

Bladder dysfunction

41
Q

How do symptoms expressed due to UTI differ depending on the age of the child?

A

Neonates - mainly fever, vomiting, lethargy and irratibility (systemic symptoms)

Children - Frequency, dysuria, abdominal pain or tenderness

42
Q

What nephrological concerns may be raised in a child with a UTI?

A

Vesicoureteral reflux causing scarring and damage to the kidneys

43
Q

What investigations may be done on a child experiencing recurrent UTI’s or UTI’s that are affecting the upper urinary tract?

A

USS (shows kidney structure)

DMSA (isotope scan - scarring & function of kidneys)

Micturating cytso-urethrogram MAG 3 scan (requires cathterization and active micturition - dynamic)

44
Q

How are UTI’s treated?

A

Lower tract - 3 days of antibiotics

Upper tract - 7 days of antibiotics

Prophylaxis - fluids, hygeine and avoiding constipation

45
Q

How does blood pressure change with age and height of the child? What criteria is necessary for a child to be diagnosed with high blood pressure?

A

It increases

The child needs to have been found to have BP in the upper 95th percentile on at least 3 occasions

46
Q

In children with high blood pressure what would you prescribe in order to improve their renal outlook?

A

ACE inhibitor

Maintaining BP in the 50th percentile improves renal outcome

47
Q

What are some supplementations that may be required if a child is suffering from CKD regarding acid base balance and endocrine function?

A

Bicarbonate supplementation

Erythropoeitin supplementation

48
Q

What treatment may be needed to prevent metabolic bone disease in children with CKD?

A

Low phosphate diets (lowers PTH)

Phosphate binders (to keep phosphate low)

Active vitamin D supplementation (to counteract PTH)

May require growth hormone supplementation if PTH is still too high to allow for normal growth

49
Q

If a child has suffered from an acute kidney injury what are some important steps to prevent them from developing chronic kidney disease?

A

Hypertension and proteinuria monitoring

Prevention of UTI’s

Preservation of normal bladder function