paediatric nephrology Flashcards

1
Q

neonate GFR

A

20-30

age 2yrs equals adults 90-120

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2
Q

5 kidney functions

A
waste handling: urea, creatinine 
water handling 
salt balance
acid base control
endocrine e.g. renin aldosterone angiotensin system
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3
Q

glomerular filtration barrier: endothelial cell

A

fenestrated

vulnerable to immune mediated injury

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4
Q

glomerular filtration barrier: glomerular basement membrane

A

2 proteins: TIV collagen and laminin

synthesis from podocytes and endothelial cells

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5
Q

glomerular filtration barrier: mesangial cells

A

smooth muscle like pericytes embedded in basement membrane

glomerular structural support
regulates blood flow of glomerular capillaries

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6
Q

what does proteinuria signify

A

glomerular injury

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7
Q

acquired glomerulopathy: epithelial cell (podocyte) affected

A

minimal change disease

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8
Q

acquired glomerulopathy: basement membrane affected

A

post infectious glomerulonephritis

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9
Q

acquired glomerulopathy: endothelial cells affected

A

post infectious glomerulonephritis

haemolytic uraemic syndrome

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10
Q

acquired glomerulopathy: mesangial cells affected

A

HSP nephritis

IgA nephropathy

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11
Q

congenital glomerulopathy: podocyte cytoskeletal integrity affected

A

congenital nephrotic syndrome

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12
Q

congenital glomerulopathy: basement membrane proteins affected

A

alport syndrome

thin basement membrane disease (AD)

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13
Q

congenital glomerulopathy: endothelial/microvascular integrity

A

membranoproliferative glomerulonephritis

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14
Q

ways of measuring proteinuria

A

dipstix - measures concentration (if 3+ then abnormal)

protein creatinine ratio (early morning urine = best)

24hr urine collection (gold standard)

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15
Q

normal and nephrotic range of protein creatinine ratio

A

normal = <20mg/mmol

nephrotic range = >250mg/mmol

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16
Q

24hr urine collection: normal and nephrotic range

A

normal <60mg/m^2/24hs

nephrotic range >1g/m^2/24hs

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17
Q

what is nephrotic synrome

A

nephrotic range protein proteinuria

causing hypoalbuminaemia and oedema

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18
Q

examination findings nephrotic syndrome

A
inflated weight 
pale
periorbital oedema 
pitting oedema legs 
ascited
pleural effusions
frothy urine 

normotensive, hypertensive or hypotensive

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19
Q

typical features of minimal change disease

A

age 1-10
normotensive
no frank haematuria
normal renal functiom

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20
Q

atypical features of minimal change disease

A

suggestions of autoimmune disease
abnormal renal function
steroid resistance

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21
Q

nephrotic syndrome treatment

A

if typical features = prednisolone 8wks

or glucocorticoids

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22
Q

side effects of high dose glucocorticoids that are more common in kids

A
behaviour
mood liability
infection risk
GI distress - more acidic 
sleep disturbance
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23
Q

steroid resistant nephrotic syndrome causes

A

acquired: focal segmental glomeruloscresosis
congenital: NPHS1 - nephrin, NPHS 2 - podocin, podocyte loss

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24
Q

causes of haematuria

A
clotting disorders
glomerulonephritis
tumours - nephroblastoma 
cysts 
malignancies
stones
UTIs
trauma
urethritis
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25
nephritic syndrome
clinical diagnosis - describes glomerulonephritis haematuria and proteinuria reduced GFR intrarenal cause of AKI
26
typical microorganism causing acute post-infectious glomerulonephritis
group a strep
27
diagnosing acute post-infectious glomerulonephritis
bacterial culture positive anti-streptolysin O titre low C3
28
treatment acute post-infectious glomerulonephritis
antibiotics support renal functions - electrolyte/acid base overload/htn - diuretics
29
IgA nephropathy
most common glomerlonephritis 1-2 days after UTI, usually older kids/adults
30
clinical features IgA nephropathy
recurrent macroscopic haematuria +/- chronic microscopic haematuria varying degree of proteinuria
31
treating IgA nephropathy
mild: proteinuria with ACEi mod - severe: immunosuppression
32
henoch schonlein purpura IgA related vasculitis (aka HSP nephritis) clinical diagnosis
mandatory palpable purpura one of - abdo pain - renal involvement - arthritis/arthralgia - biopsy: IgA deposition
33
IgA vasculitis
small vessel vasculitis overlapping with IgA nephropathy
34
IgA vasculitis features
1-3days post trigger - viral, streptococcus nephritis - mesangial cell injury
35
x
x
36
acute kidney injury
abrupt loss of kidney function | retention of urea and other waste products and dysregulation of extracellular volume and electrolytes
37
AKI features - kids
anuria/oliguria (<0.5ml/kg/hr) htn with fluid overload rapid rise in plasma creatinine
38
AKI diagnostic definition
serum creatinine >1.5x age specific reference creatinine (or previous baseline if known) urine output <0.5ml/kg >8hrs
39
AKI warning score: AKI 1
creatinine >1.5-2x reference
40
AKI warning score: AKI 2
creatinine 2-3x reference
41
AKI warning score: AKI 3
creatinine >3x reference
42
AKI management
3 Ms monitor: paediatric early warning scores, urine output, weight maintain: good hydration, electrolytes, acid-base minimise: drugs
43
causes of AKI: pre-renal
perfusion problem volume depletion renal hypoperfusion: drugs, renal a. stenosis, hepato-renal syndrome haemorrhage hypotension
44
causes of AKI: intrinsic renal problem
glomerular disease: haemolytic uraemic syndrome, glomerulonephritis tubular injury: acute tubular necrosis interstisial nephritis: NSAID, autoimmune
45
causes of AKI: post-renal
obstructive uropathies
46
haemolytic uraemic syndrome
haemolysis - packed cell volume <30%, hg level <10 and fragmented erythrocytes on blood film thrombocytopenia - platelet count <150 AKI - inc serum creatinine, GFR <80
47
typical HUS causes
post (bloody) diarrhoea entero-haemorrhagic e.coli - produce shiga toxic other - pneumococcal infection, drugs
48
atypical HUS
genetic condition resulting in overactivation of alternative complement pathway
49
HUS triad
microangiopathic haemolytic anaemia thrombocytopenia AKI/acute renal failure
50
management of HUS
monitor: 5 kidney functions maintain: IV normal saline and fluid, renal replacement therapy minimise: no antibiotics/NSAIDs
51
longterm consequences of AKI
htn proteinuria monitoring evolution to CKD
52
CKD causes: congenital
reflux nephropathy dysplasia obstructive uropathy
53
CKD causes: hereditary
cystic kidney disease | cystinosis
54
when do CKD signs/symptoms start to show
<60% renal function (stage 3)
55
presentation of CKD
inc urea: lose appetite, weight loss, itch, cardio/neuro functio water handling: polyuria or oliguria salt/acid base - lethargy, poor growth endocrine - lethargy, hyper-/hypo-tensive, anaemic bladder: UTIs
56
most common symptoms of UTI in neonates
fever vomiting lethargy irritability
57
diagnosing UTI
dipstix - white cells, nitrtites microscopy - pyuria, bacturia culture >10^5 colony forming units
58
treatment UTI
lower tract - 3 days oral antibiotic upper tract - antibiotic 7/10 days, oral is systemically well
59
radiology diagnosing UTI
USS DMSA isotope scan micturition cysto-urethrogram
60
factors affecting progression of CKD
``` late referral htn proteinuria high intake protein, phosphate, salt bone health acidosis recurrent UTIs ```
61
IgA nephropathy vs post-strep GN
IgA nephropathy soon after or alongside URTI PSGN - week(s) after
62
transient proteinuria
proteinura following acute event e.g. seizure, infection | resolves within 24hrs/when underlying cause treate
63
orthostatic proteinuria
asymptomatic, well increasing proteinuria throughout day adoescents AM and PM protein dip
64
HSP management
glucocorticoid therapy immunosuppression long term HTN and proteinuria screening