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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
What occurs in FSGS?
Podocyte loss | Progressive inflammation and sclerosis
26
When should you investigate haematuria?
Macroscopic or microscopic > trace on 2 occasions
27
What is haemaglobulinuria?
Stix positive & microscopy negative
28
What does persistent haematuria and proteinuria indicate?
Glomerular disease
29
What are the causes of macroscopic haematuria?
``` Glomerulonephritis (post-infectious GN, IgA/HSP), SLE UTI Trauma Stones HUS etc. ```
30
What are the causes of microscopic haematuria?
``` GN (post-infectious GN, IgA, HSP), SLE, ANCA +ve vasculitis UTI Trauma Stones/hypercalcuria HUS Sickle cell dx etc. ```
31
What investigations do you want to do in haematuria?
``` Dipstix Red cells on microscopy Creatnine & electrolytes FBC Albumin Urine culture ```
32
What features are consistent with nephritic syndrome?
``` Reduced GFR Oliguria Fluid overload (raised JVP, oedema) HTN Haematuria Proteinuria ```
33
What are the causes of nephritic GN?
``` Post-infectious GN HSP/IgA nephropathy Membranoproliferative GN Lupus nephritis ANCA positive vasculitis ```
34
How do you investigate nephritic GN further?
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
35
What is ASO?
Antibody against exotoxin produced by group A (C and G) strep
36
What tends to cause post-infectious glomerulonephritis?
Usually group A (beta-haemolytic) strep Infection in throat - 7-10 days or skin - 2-4 days
37
What is thought to cause post-infectious GN?
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
What is the prognosis of acute post-infectious GN?
Self-limiting
39
How do you diagnose post-infectious GN?
Bacterial culture +ve ASOT Low C3 - ensure this normalises - if it doesn't MPGN is differential diagnosis
40
How do you Rx post-infectious GN?
Antibiotics (penicillin for GAS) Support renal function Overload/HTN (diuretics)
41
What is the most common GN?
IgA nephropathy
42
What are typical features of IgA nephropathy?
1-2 days after URTI Usually adults/older children Recurrent macroscopic haematuria +/- chronic microscopic haematuria Varying degree of proteinuria
43
What is the pathogenesis of IgA nephropathy?
Defective (galactose deficient) IgA clump together and deposit in the kidney --> glomerulonephritis
44
How do you diagnose IgA nephropathy?
Clinically - +ve autoimmune workup, normal complement | Confirmation biopsy
45
How do you Rx IgA nephropathy?
Mild disease - proteinuria with ACEi | Mild-mod: immunosuppression
46
What is the prognosis of IgA nephropathy?
25% end stage renal failure 10yrs post diagnosis
47
What is Henoch schonlein purpura IgA related vasculitis?
``` 5-15yo Mandatory palpable purpura + 1 of: Abdominal pain Renal involvement Arthritis/arthralgia Biopsy - IgA deposition ``` Basically it is IgA vasculitis with nephritis
48
What tends to cause IgA vasculitis?
1-3 days post-trigger, e.g. viral URTI (strep, drugs)
49
How long does IgA vasculitis tend to last?
4-6 weeks
50
In IgA vasculitis what is the nephritis a result of?
Mesangial injury
51
How do you Rx IgA vasculitis?
Steroids Immunosupression (if mod-severe renal dx) - cyclophosphamide/prednisolone Long term - HTN & proteinuria
52
Define AKI
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
What findings are typical of AKI?
Anuria/oliguria (<0.5l/kg/hr) HTN with fluid overload Rapid rise in plasma creatinine
54
What are the classifications of AKI?
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
What is involved in the Mx of AKI?
Monitor - UO, PEWs, BP, weight Maintain - good hydration Minimise - drugs
56
What are the pre-renal causes of AKI?
Perfusion problems | E.g. volume depletion, hypotension, drugs, renal artery stenosis
57
What are the intrinsic renal problem causes of AKI?
Glomerular disease - HUS, GN Tubular injury - ATN Interstitial nephritis - NSAIDs, autoimmune
58
What is ATN?
Acute tubular necrosis | Consequence of hypoperfusion/drugs
59
What are the post-renal causes of AKI?
Obstructive
60
What does HUS tend to follow after?
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
What % of children with E. coli 0157 infection will develop HUS?
15%
62
What does E. coli 0157 colonise?
Sheep/cattle
63
What is the triad of HUS?
Microangiopathic haemolytic anaemia Thrombocytopenia AKI
64
How do you Rx HUS?
Monitor 5 kidney functions - HTN, IV saline/fluid, RRT | Don't use antibiotics
65
What are the complications of HUS?
Seizures, acute abdomen, diabetes, adrenal crisis | May evolve into CKD
66
What are the causes for paediatric CKD?
Congenital abnormalities of the kidney & urinary tract (e.g. reflux nephropathy, dysplasia, obstructive uropathy) Hereditary conditions, e.g. cystinosis, cystic kidney disease
67
CAKUT may not be isolated. What things may it be related to?
Turner Trisomy 21 Branchio-oto-renal Prune belly syndrome (absence of abdominal muscles, bilateral cryptorchidism, urinary tract malformations)
68
Under what GFR do you start to get signs and symptoms of CKD?
60 (large renal reserve) CKD 2 is asymptomatic At CKD 5 think about RRT
69
What is the presentation of CKD?
``` Pruritus Encephalitis Polyuric (tubular damage) Salt wasting - low Na, K usually high Bladder dysfunction Anaemia HTN - RAAS raised ETC ```
70
Define UTI
Symptomatic + bacteria culture from MSSU or any growth on suprapubic aspiration/catheter
71
What are the symptoms of UTIs?
Neonates - fever, vomiting, lethargy, irritability, poor feeding Children - fever, dysuria, frequency, abdominal pain, poor feeding, vomiting, nocturnal enuresis
72
How can you obtain urine specimens from children?
Clean catch urine/MSSU Collection pads/urine pags Catheters/suprapubic aspiration if sick
73
How can you make a diagnosis of a UTI?
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
How is VUR graded?
``` 1 - ureter only 2 - ureter, pelvis, calyces 3 - dilatation ureter 4 - moderate dilatation of ureter 5 - gross dilatation/tortuosity ```
75
When do you Rx UTIs?
<6m Abnormal anatomy/bladder Septic presentation Recurrent UTIs
76
In which children do you screen in UTIs?
Screen in those at risk of progressive scarring (reflux nephropathy) Capture those with renal dysplasia
77
What investigations can you do to look at the kidneys?
USS - structure DMSA - scarring/function Micturating cystourethrogram/MAG3 - dynamic
78
How do you Rx UTIs in children?
Lower tract - oral antibx 3 days (trimethoprim, coamoxiclav, cephalosporin) Upper tract/pyelonephritis - antibx for 7-10 days PO if systemically well
79
How do you prevent UTIs?
Good hygiene Avoid constipation Keep hydrated
80
What factors affect progression of CKD?
``` Late referral HTN Proteinuria High intake protein, phosphate, salt Bone health - PTH, phosphate, Vit D Acidosis Recurrent UTIs ```
81
How is CKD classified?
KDIGO 2012
82
How do you measure BP in a child?
Doppler Oscillometry Sphingmanometer 24h ambulatory BP monitoring
83
What factors affect BP?
Sex, age, height
84
Define HTN
95th centile over above Borderline is 90-95th centile
85
How do you Mx CKD?
``` 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
What heart problems do you get in CKD?
Accelerated atherosclerosis

Paediatrics (35 decks)

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