paediatric nephrology Flashcards
neonate GFR
20-30
age 2yrs equals adults 90-120
5 kidney functions
waste handling: urea, creatinine water handling salt balance acid base control endocrine e.g. renin aldosterone angiotensin system
glomerular filtration barrier: endothelial cell
fenestrated
vulnerable to immune mediated injury
glomerular filtration barrier: glomerular basement membrane
2 proteins: TIV collagen and laminin
synthesis from podocytes and endothelial cells
glomerular filtration barrier: mesangial cells
smooth muscle like pericytes embedded in basement membrane
glomerular structural support
regulates blood flow of glomerular capillaries
what does proteinuria signify
glomerular injury
acquired glomerulopathy: epithelial cell (podocyte) affected
minimal change disease
acquired glomerulopathy: basement membrane affected
post infectious glomerulonephritis
acquired glomerulopathy: endothelial cells affected
post infectious glomerulonephritis
haemolytic uraemic syndrome
acquired glomerulopathy: mesangial cells affected
HSP nephritis
IgA nephropathy
congenital glomerulopathy: podocyte cytoskeletal integrity affected
congenital nephrotic syndrome
congenital glomerulopathy: basement membrane proteins affected
alport syndrome
thin basement membrane disease (AD)
congenital glomerulopathy: endothelial/microvascular integrity
membranoproliferative glomerulonephritis
ways of measuring proteinuria
dipstix - measures concentration (if 3+ then abnormal)
protein creatinine ratio (early morning urine = best)
24hr urine collection (gold standard)
normal and nephrotic range of protein creatinine ratio
normal = <20mg/mmol
nephrotic range = >250mg/mmol
24hr urine collection: normal and nephrotic range
normal <60mg/m^2/24hs
nephrotic range >1g/m^2/24hs
what is nephrotic synrome
nephrotic range protein proteinuria
causing hypoalbuminaemia and oedema
examination findings nephrotic syndrome
inflated weight pale periorbital oedema pitting oedema legs ascited pleural effusions frothy urine
normotensive, hypertensive or hypotensive
typical features of minimal change disease
age 1-10
normotensive
no frank haematuria
normal renal functiom
atypical features of minimal change disease
suggestions of autoimmune disease
abnormal renal function
steroid resistance
nephrotic syndrome treatment
if typical features = prednisolone 8wks
or glucocorticoids
side effects of high dose glucocorticoids that are more common in kids
behaviour mood liability infection risk GI distress - more acidic sleep disturbance
steroid resistant nephrotic syndrome causes
acquired: focal segmental glomeruloscresosis
congenital: NPHS1 - nephrin, NPHS 2 - podocin, podocyte loss
causes of haematuria
clotting disorders glomerulonephritis tumours - nephroblastoma cysts malignancies stones UTIs trauma urethritis
nephritic syndrome
clinical diagnosis - describes glomerulonephritis
haematuria and proteinuria
reduced GFR
intrarenal cause of AKI
typical microorganism causing acute post-infectious glomerulonephritis
group a strep
diagnosing acute post-infectious glomerulonephritis
bacterial culture
positive anti-streptolysin O titre
low C3
treatment acute post-infectious glomerulonephritis
antibiotics
support renal functions - electrolyte/acid base
overload/htn - diuretics
IgA nephropathy
most common glomerlonephritis
1-2 days after UTI, usually older kids/adults
clinical features IgA nephropathy
recurrent macroscopic haematuria
+/- chronic microscopic haematuria
varying degree of proteinuria
treating IgA nephropathy
mild: proteinuria with ACEi
mod - severe: immunosuppression
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
IgA vasculitis
small vessel vasculitis
overlapping with IgA nephropathy
IgA vasculitis features
1-3days post trigger - viral, streptococcus
nephritis - mesangial cell injury
x
x
acute kidney injury
abrupt loss of kidney function
retention of urea and other waste products and dysregulation of extracellular volume and electrolytes
AKI features - kids
anuria/oliguria (<0.5ml/kg/hr)
htn with fluid overload
rapid rise in plasma creatinine
AKI diagnostic definition
serum creatinine >1.5x age specific reference creatinine (or previous baseline if known)
urine output <0.5ml/kg >8hrs
AKI warning score: AKI 1
creatinine >1.5-2x reference
AKI warning score: AKI 2
creatinine 2-3x reference
AKI warning score: AKI 3
creatinine >3x reference
AKI management
3 Ms
monitor: paediatric early warning scores, urine output, weight
maintain: good hydration, electrolytes, acid-base
minimise: drugs
causes of AKI: pre-renal
perfusion problem
volume depletion
renal hypoperfusion: drugs, renal a. stenosis, hepato-renal syndrome
haemorrhage
hypotension
causes of AKI: intrinsic renal problem
glomerular disease: haemolytic uraemic syndrome, glomerulonephritis
tubular injury: acute tubular necrosis
interstisial nephritis: NSAID, autoimmune
causes of AKI: post-renal
obstructive uropathies
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
typical HUS causes
post (bloody) diarrhoea
entero-haemorrhagic e.coli - produce shiga toxic
other - pneumococcal infection, drugs
atypical HUS
genetic condition resulting in overactivation of alternative complement pathway
HUS triad
microangiopathic haemolytic anaemia
thrombocytopenia
AKI/acute renal failure
management of HUS
monitor: 5 kidney functions
maintain: IV normal saline and fluid, renal replacement therapy
minimise: no antibiotics/NSAIDs
longterm consequences of AKI
htn
proteinuria monitoring
evolution to CKD
CKD causes: congenital
reflux nephropathy
dysplasia
obstructive uropathy
CKD causes: hereditary
cystic kidney disease
cystinosis
when do CKD signs/symptoms start to show
<60% renal function (stage 3)
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
most common symptoms of UTI in neonates
fever
vomiting
lethargy
irritability
diagnosing UTI
dipstix - white cells, nitrtites
microscopy - pyuria, bacturia
culture >10^5 colony forming units
treatment UTI
lower tract - 3 days oral antibiotic
upper tract - antibiotic 7/10 days, oral is systemically well
radiology diagnosing UTI
USS
DMSA isotope scan
micturition cysto-urethrogram
factors affecting progression of CKD
late referral htn proteinuria high intake protein, phosphate, salt bone health acidosis recurrent UTIs
IgA nephropathy vs post-strep GN
IgA nephropathy soon after or alongside URTI
PSGN - week(s) after
transient proteinuria
proteinura following acute event e.g. seizure, infection
resolves within 24hrs/when underlying cause treate
orthostatic proteinuria
asymptomatic, well
increasing proteinuria throughout day
adoescents
AM and PM protein dip
HSP management
glucocorticoid therapy
immunosuppression
long term HTN and proteinuria screening
