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Paediatrics > Nephrology > Flashcards

Nephrology Flashcards

1
Q

What is the GFR of a neonate?

A

20-30ml/min/1.73m2

Becomes adult GFR at age 2

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2
Q

What are the 5 major functions of the kidney?

A 
Waste handling - urea/creatinine 
Water handling 
Salt balance - K/Na/Ca/phosphate
Acid base control - bicarbonate 
Endocrine - RAAS/PTH/activation of vit D/EPO
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3
Q

What are the three layers composing the glomerular filtration barrier?

A

Endothelial cell layer: fenestrated to allow all components of plasma apart from RCs through

Glomerular basal lamina/basement membrane: blocks large proteins getting through

Podocytes: slits prevent medium sized proteins getting through

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4
Q

What is the GBM composed of?

A

Type IV collage and laminin

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5
Q

What are podocytes composed of?

A

Proteins (podocin/nephrin)

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6
Q

What are the mesangial cells?

A

Glomerular structural support
Embedded within GMB
Regulates blood flow of glomerular capillaries

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7
Q

What signifies glomerular injury?

A

PROTEINURIA

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8
Q

What symptoms are more down the nephritic end of the spectrum of renal damage?

A

Increasing haematuria

Intravascular overload

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9
Q

What symptoms are more down the nephrotic end of the spectrum of renal damage?

A

Increasing proteinuria

Intravascular depletion

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10
Q

What are the congenital glomerulopathies?

A

Podocyte cytoskeleton integrity disorders - defects in podocin/nephrin proteins (AR)

Basement membrane defects - Alports (XL), thin basement membrane dx (AD)

Endothelial/microvascular integrity - complete regulatory proteins (MPGN)

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11
Q

What occurs in nephrotic syndrome?

A

Proteinuria
Hypoalbuminaemia
Oedema

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12
Q

Why do you get oedema in hypoalbuminaemia?

A

Not enough oncotic pressure to balance out hydrostatic forces driving water out of cells

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13
Q

What is the appearance of proteinuria?

A

Frothy urine

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14
Q

How do you test for proteinuria?

A

Dipstix - 3+ or more usually abnormal

Protein creatinine ratio - early morning urine best (normal Pr:Cr ratio <20mg/mmol, nephrotic range >250mg/mmol)
24hr urine collection is gold standard but not really practical

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15
Q

Why would someone with nephrotic syndrome have low urine sodium?

A

They are trying to retain sodium to try and retain IV volume

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16
Q

Most kids with nephrotic syndrome will have what?

A

Minimal change disease (idiopathic)

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17
Q

What are the typical features of MCD?

A 
2-5yrs
Nephrotic syndrome 
Normal BP 
Resolving microscopic haematuria
Normal renal function 
Steroid responsive
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18
Q

When should you biopsy in suspected MCD?

A

Only if suggestion of autoimmune dx, abnormal renal function, steroid resistance

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19
Q

How do you Rx nephrotic syndrome (MCD)?

A

Prednisolone (8wks)

If MCD should go into remission within 2 weeks

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20
Q

What must you warn parents of when putting kids on prednisolone?

A

SEs - esp. behavioural problems (irritable, moody), GI distress, may impact growth if long term

Check varicella status and pneumococcal vaccination as is immunosupressive

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21
Q

What is the pathogenesis of MCD?

A

T cell and cytokine mediated damage of the GBM and podocytes –> albumin can leak through and is excreted

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22
Q

What is the prognosis for MCD?

A

80% long term remission

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23
Q

If MCD is steroid resistant what can you treat it with?

A

Probably more FSGS if steroid resistant

Can Rx with cyclosporin, tacrolimus, rituximab etc.

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24
Q

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

A

Focal segmental glomeruloscerosis (FSGS)

Infant presentations may be congenital dx e.g. NPHS1, NPHS2

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25
Q

What occurs in FSGS?

A

Podocyte loss

Progressive inflammation and sclerosis

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26
Q

When should you investigate haematuria?

A

Macroscopic or microscopic > trace on 2 occasions

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27
Q

What is haemaglobulinuria?

A

Stix positive & microscopy negative

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28
Q

What does persistent haematuria and proteinuria indicate?

A

Glomerular disease

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29
Q

What are the causes of macroscopic haematuria?

A 
Glomerulonephritis (post-infectious GN, IgA/HSP), SLE
UTI
Trauma
Stones
HUS etc.
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30
Q

What are the causes of microscopic haematuria?

A 
GN (post-infectious GN, IgA, HSP), SLE, ANCA +ve vasculitis
UTI
Trauma
Stones/hypercalcuria 
HUS 
Sickle cell dx 
etc.
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31
Q

What investigations do you want to do in haematuria?

A 
Dipstix 
Red cells on microscopy 
Creatnine &amp; electrolytes
FBC
Albumin 
Urine culture
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32
Q

What features are consistent with nephritic syndrome?

A 
Reduced GFR
Oliguria 
Fluid overload (raised JVP, oedema)
HTN
Haematuria
Proteinuria
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33
Q

What are the causes of nephritic GN?

A 
Post-infectious GN 
HSP/IgA nephropathy
Membranoproliferative GN 
Lupus nephritis
ANCA positive vasculitis
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34
Q

How do you investigate nephritic GN further?

A

Renal USS
Anti-streptolysin O titre
Throat swab for GAS
Complete (C3 low, normal C4 as C3 being used in PIGN)

Immune dx - ANA/ANCA
Biopsy not required if ASOT high, nephritic syndrome and +ve throat swab - you know its post-infectious GN

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35
Q

What is ASO?

A

Antibody against exotoxin produced by group A (C and G) strep

36
Q

What tends to cause post-infectious glomerulonephritis?

A

Usually group A (beta-haemolytic) strep

Infection in throat - 7-10 days or skin - 2-4 days

37
Q

What is thought to cause post-infectious GN?

A

Glomerular trapping of circulating immune complexes

Nephrogenic antigens on strep bind specific sites in glomerulus

Either of these set up humeral/cellular immune response and activate alternative complement pathway –> AKI

38
Q

What is the prognosis of acute post-infectious GN?

A

Self-limiting

39
Q

How do you diagnose post-infectious GN?

A

Bacterial culture
+ve ASOT
Low C3 - ensure this normalises - if it doesn’t MPGN is differential diagnosis

40
Q

How do you Rx post-infectious GN?

A

Antibiotics (penicillin for GAS)
Support renal function
Overload/HTN (diuretics)

41
Q

What is the most common GN?

A

IgA nephropathy

42
Q

What are typical features of IgA nephropathy?

A

1-2 days after URTI
Usually adults/older children
Recurrent macroscopic haematuria +/- chronic microscopic haematuria
Varying degree of proteinuria

43
Q

What is the pathogenesis of IgA nephropathy?

A

Defective (galactose deficient) IgA clump together and deposit in the kidney –> glomerulonephritis

44
Q

How do you diagnose IgA nephropathy?

A

Clinically - +ve autoimmune workup, normal complement

Confirmation biopsy

45
Q

How do you Rx IgA nephropathy?

A

Mild disease - proteinuria with ACEi

Mild-mod: immunosuppression

46
Q

What is the prognosis of IgA nephropathy?

A

25% end stage renal failure 10yrs post diagnosis

47
Q

What is Henoch schonlein purpura IgA related vasculitis?

A 
5-15yo
Mandatory palpable purpura + 1 of:
Abdominal pain 
Renal involvement
Arthritis/arthralgia
Biopsy - IgA deposition

Basically it is IgA vasculitis with nephritis

48
Q

What tends to cause IgA vasculitis?

A

1-3 days post-trigger, e.g. viral URTI (strep, drugs)

49
Q

How long does IgA vasculitis tend to last?

A

4-6 weeks

50
Q

In IgA vasculitis what is the nephritis a result of?

A

Mesangial injury

51
Q

How do you Rx IgA vasculitis?

A

Steroids
Immunosupression (if mod-severe renal dx) - cyclophosphamide/prednisolone
Long term - HTN & proteinuria

52
Q

Define AKI

A

Abrupt loss of kidney function –> retention of urea & other nitrogenous waste productions & dysregulation of extracellular volume & electrolytes

Serum creatinine >1.5x age specific reference/prev. baseline
UO <0.5ml/kg for 8h

53
Q

What findings are typical of AKI?

A

Anuria/oliguria (<0.5l/kg/hr)
HTN with fluid overload
Rapid rise in plasma creatinine

54
Q

What are the classifications of AKI?

A

AKI 1 - Cr >1.5-2x reference creatinine/ULRI

AKI 2 - Cr 2-3x reference creatinine/ULRI

AKI 3 - Cr >3x reference creatinine/ULRI

55
Q

What is involved in the Mx of AKI?

A

Monitor - UO, PEWs, BP, weight
Maintain - good hydration
Minimise - drugs

56
Q

What are the pre-renal causes of AKI?

A

Perfusion problems

E.g. volume depletion, hypotension, drugs, renal artery stenosis

57
Q

What are the intrinsic renal problem causes of AKI?

A

Glomerular disease - HUS, GN
Tubular injury - ATN
Interstitial nephritis - NSAIDs, autoimmune

58
Q

What is ATN?

A

Acute tubular necrosis

Consequence of hypoperfusion/drugs

59
Q

What are the post-renal causes of AKI?

A

Obstructive

60
Q

What does HUS tend to follow after?

A

Entero-haemorrhagic E. coli (verocytotoxin/shigella toxin) - E. coli 0157 - tends to be 14 days after onset of diarrhoea
Other causes: pneumococcal infection, drugs

61
Q

What % of children with E. coli 0157 infection will develop HUS?

A

15%

62
Q

What does E. coli 0157 colonise?

A

Sheep/cattle

63
Q

What is the triad of HUS?

A

Microangiopathic haemolytic anaemia
Thrombocytopenia
AKI

64
Q

How do you Rx HUS?

A

Monitor 5 kidney functions - HTN, IV saline/fluid, RRT

Don’t use antibiotics

65
Q

What are the complications of HUS?

A

Seizures, acute abdomen, diabetes, adrenal crisis

May evolve into CKD

66
Q

What are the causes for paediatric CKD?

A

Congenital abnormalities of the kidney & urinary tract (e.g. reflux nephropathy, dysplasia, obstructive uropathy)

Hereditary conditions, e.g. cystinosis, cystic kidney disease

67
Q

CAKUT may not be isolated. What things may it be related to?

A

Turner
Trisomy 21
Branchio-oto-renal
Prune belly syndrome (absence of abdominal muscles, bilateral cryptorchidism, urinary tract malformations)

68
Q

Under what GFR do you start to get signs and symptoms of CKD?

A

60 (large renal reserve)
CKD 2 is asymptomatic
At CKD 5 think about RRT

69
Q

What is the presentation of CKD?

A 
Pruritus
Encephalitis
Polyuric (tubular damage) 
Salt wasting - low Na, K usually high 
Bladder dysfunction 
Anaemia
HTN - RAAS raised 
ETC
70
Q

Define UTI

A

Symptomatic + bacteria culture from MSSU or any growth on suprapubic aspiration/catheter

71
Q

What are the symptoms of UTIs?

A

Neonates - fever, vomiting, lethargy, irritability, poor feeding

Children - fever, dysuria, frequency, abdominal pain, poor feeding, vomiting, nocturnal enuresis

72
Q

How can you obtain urine specimens from children?

A

Clean catch urine/MSSU
Collection pads/urine pags
Catheters/suprapubic aspiration if sick

73
Q

How can you make a diagnosis of a UTI?

A

Dipstix - leucocytes & nitrates may indicate UTI (but unreliable under 2y)

Microscopy - pyuria >10 WBC per cubic mm, bacturia

Culture >10^5 colony forming units (E. coli)

74
Q

How is VUR graded?

A 
1 - ureter only
2 - ureter, pelvis, calyces
3 - dilatation ureter
4 - moderate dilatation of ureter
5 - gross dilatation/tortuosity
75
Q

When do you Rx UTIs?

A

<6m
Abnormal anatomy/bladder
Septic presentation
Recurrent UTIs

76
Q

In which children do you screen in UTIs?

A

Screen in those at risk of progressive scarring (reflux nephropathy)
Capture those with renal dysplasia

77
Q

What investigations can you do to look at the kidneys?

A

USS - structure
DMSA - scarring/function
Micturating cystourethrogram/MAG3 - dynamic

78
Q

How do you Rx UTIs in children?

A

Lower tract - oral antibx 3 days (trimethoprim, coamoxiclav, cephalosporin)
Upper tract/pyelonephritis - antibx for 7-10 days PO if systemically well

79
Q

How do you prevent UTIs?

A

Good hygiene
Avoid constipation
Keep hydrated

80
Q

What factors affect progression of CKD?

A 
Late referral 
HTN 
Proteinuria
High intake protein, phosphate, salt
Bone health - PTH, phosphate, Vit D 
Acidosis
Recurrent UTIs
81
Q

How is CKD classified?

A

KDIGO 2012

82
Q

How do you measure BP in a child?

A

Doppler
Oscillometry
Sphingmanometer

24h ambulatory BP monitoring

83
Q

What factors affect BP?

A

Sex, age, height

84
Q

Define HTN

A

95th centile over above

Borderline is 90-95th centile

85
Q

How do you Mx CKD?

A 
Watch protein intake
Salt, K restriction 
EPO for anaemia
ACEi for HTN 
etc.

For bone dx - low phosphate diet, phosphate binders, active vit D
If poor on-going growth - HGH

86
Q

What heart problems do you get in CKD?

A

Accelerated atherosclerosis

Paediatrics (35 decks)

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