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Y3 paediatrics > paediatric nephrology > Flashcards

paediatric nephrology Flashcards

1
Q

CO to nephron

A

recieves 25% CO/min

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2
Q

GFR in children

A

neonate 20-30ml/min/1.73m2

2yrs - equivalent to adult, 90-120

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3
Q

GFR in children

A

neonate 20-30ml/min/1.73m

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4
Q

label the nephron

A
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5
Q

what are the 5 functions of the kidney

A
  1. waste handling
  2. water handling
  3. salt balance
  4. acid base control
  5. endocrine - RBC, BP, bone health
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6
Q

components of the glomerular filtration barrier

A

endothelial cell:

  • fenestrated
  • vulnerable to immune mediated injury

GBM

  • type IV collagen and laminin
  • synthesis from podocytes and endothelial cells
  • mesangial cells playing a role in turnover

podocyte

  • proteins - podocin, nephrin

mesangial cells

  • glomerular structural support
  • embedded in GBM
  • regulates blood flow of the glomerular capillaries
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7
Q

how does the glomerular filtration barrier work

A
  • works like a sieve
  • larger holes = greater leak
  • glomerular filtration within the capillary lumen
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8
Q

how do patients present with glomerulopathy

A

proteinuria and haematuria

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9
Q

clinical presentation of glomerulopathy

A
  • blood and protein in varying amounts dictate clinical presentation and suggest diagnosis
  • BUT injury to one part of the GFB affects the other components
  • proteinuria signifies glomerular injury
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10
Q

what do different amounts of haematuria and proteinuria indicate

A
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11
Q

how common is acquired glomerulopathy

A

common

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12
Q

which components are affected in the different types of acquired glomerulopathies

A

epithelial cell (podocyte) - minimal change disease (nephrotic)

basement membrane - post infectious glomerulonephritis (nephritic)

endothelial cell - PIGN, haemolytic uraemic syndrome (nephritic)

mesangial cell - HSP/IgA nephropathy (mixed picture)

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13
Q

how common are congenital glomerulopathies

A

rare

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14
Q

layers involved in congenital glomerulopathies

A

podocyte skeletal integrity - congenital nephrotic syndrome (podocin - AR, nephrin - AR)

basement membrane proteins - alport syndrome (XL), thin basement membrane disease (AD)

endothelial/microvascular integrity - complement regulatory proteins (MPGN)

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15
Q

how much proteinuria is too much

A

dipstix

  • measures concentration
  • ≥3+ usually abnormal
  • false +ves/-ves

protein creatinine ratio (practical)

  • early morning urine best
  • normal: Pr:CR ration <20mg/mmol
  • nephrotic range: >250mg/mmol

24hr urine collection (gold standard)

  • normal <60mg/m2/24hrs
  • nephrotic range >1g/m2/24hrs
  • adults >3.5g/24hrs
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16
Q

what is nephrotic syndrome

A

nephrotic range proteinuria → hypoalbuminaemia → oedema (increasing 3rd space vol)

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17
Q

Starling’s forces

A

oncotic (osmotic) vs hydrostatic pressure

proteins hold water intravasculary

hypoalbuminaemia → 3rd space fluid loss (hydrostatic pressure drives fluid out)

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18
Q

typical presentation of nephrotic syndrome - hx

A

young child

present w/ minimal change disease or steroid sensitive nephrotic syndrome

prev/intercurrent illness e.g. gastroenteritis

short hx of oedema (face worse in AM, legs worse in PM)

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19
Q

nephrotic syndrome - features on examination

A

pallor

inflated weight

periorbital oedema, pitting oedema in lower limbs, ascites, pleural effusions

BP normal/raised/low

frothy urine

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20
Q

making a diagnosis of nephrotic syndrome

A
  • odema
  • proteinuria
    • urine dipstix - protein +++, blood ++ (not frank, 50% w/ MCD have microscopic haematuria)
    • Pr:CR - 1200mg/mmol creatinine
    • urine Na - 10mmol/l - low, holding onto water
  • bloods
    • hypoalbuminaemia - 12mg/dl (n >32)
    • normal creatinine
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21
Q

type of nephrotic syndrome and age at presentation

A

80% of children have minimal change disease

steroid resistance - focal segmental glomerulonephritis, membranoproliferative glomerulonephritis

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22
Q

typical features of minimal change disease

A

1-10y/o

normal BP

no frank haematuria - microscopic haematuria normally resolves

normal renal function

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23
Q

atypical features in MCD

A

suggestions of AI disease

abnormal renal function

steroid resistance - failure to go into remission after 4wks high dose oral steroid

  • only then consider renal biopsy
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24
Q

do we biopsy for MCD

A

no

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25
Q

treatment of nephrotic syndrome

A

if typical features - 8wks prednisolone

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26
Q

side effects from high dose glucocorticoids

A

Cushing’s syndrome

parents notice - behaviour, mood lability, sleep disturbance

infection risk - varicella status, pneumococcal vaccination, abx prophylaxis

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27
Q

features of Cushing’s syndrome - which are more common in children

A

personality change, moon face, increased susceptibility to infection, gynaecomastia, fat deposits on face and back of shoulders, growth/osteoporosis, bruising and petechiae, striae, skin thinning, amenorrhoea, hirsutism, GI distress - increased acid, thin extremities, hypertension, oedema, CNS irritability, hyperglycaemia

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28
Q

idiopathic NS in childhood

A

steroid sensitivity predicts diagnosis and prognosis

90% steroid sensitive - non relapsing, infrequently relapsing, frequently relapsing, steroid dependent → more likely to be MCD

steroid resistance → more suggestive of FSGN

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29
Q

interaction between immune system and podocytes

A
  • if we affect functioning of B and T cells you can reduce the number of relapses in nephrotic syndrome
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30
Q

outcome in nephrotic syndrome

A

relapse - 95% in 2-4wks

relapse - 80%

80% long term remission

for frequently relapsing - 2nd line immunosuppression (remember steroid toxicity - steroid dependent and >4 relapses p/a

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31
Q

steroid resistant nephrotic syndrome

A

acquired

  • focal segmental glomerulosclerosis (FSGS) - podocyte loss, progressive inflammation and sclerosis

congenital

  • infant presentations
  • NPHS1 - nephrin
  • NPHS 2 - podocin
  • podocyte loss
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32
Q

prognosis of FSGS

A

50% require renal replacement therapy in 5yrs

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33
Q

haematuria in children

A
  • macroscopic/frank - ALWAYS INVESTIGATE
  • microscopic - dipstix adequate
    • investigate if > trace on 2 occasions
    • haemoglobinuria - stix +ve and microscopy -ve
  • associated proteinuria = glomerular disease
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34
Q

causes of haematuria

A

urinary tract

  • malignancies - sarcomas
  • stones
  • UTI
  • trauma
  • urethritis

renal

  • glomerulonephritis
  • tumour - Wilm’s (nephroblastoma)
  • cysts

systemic

  • clotting disorders
    *
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35
Q

investigations in haematuria

A

bloods

  • waste accumulation (creatinine)
  • electrolytes - may seen hyponatraemia and hyperkalaemia
  • FBC - anaemia, haemolysis
  • albumin

urine

  • exclude UTI (culture)
  • any overlap w/ nephrotic syndrome
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36
Q

what is nephritic syndrome and what are the features

A

clinical diagnosis - describes glomerulonephritis

  • haematuria and proteinuria
  • reduced GFR
    • oliguria
    • fluid overload - raised JVP, oedema
  • hypertension
  • worsening renal failure → rapidly progressive GN
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37
Q

how does glomerulonephritis cause AKI

A

intrarenal cause of AKI

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38
Q

which components are affected in acquired glomerulopathy w/ frank haematuria

A

epithelial cell (podocyte) - MCD, FSGS, lupus

basement membrane - membranous glomerulopathy, MPGN, GS, PIGN

endothelial cell - PIGN, HUS, membranoproliferative glomerulonephritis, lupus, ANCA vasculitis

mesangial cell - HSP/IgA nephropathy, lupus

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39
Q

further investigations to make a diagnosis in nephritic syndrome

A

radiology - renal USS

chase most likely diagnosis - ASOT, throat swab (strep)

immunology workup - complement C3 and 4, AI diseases (ANA, ANCA)

biopsy

40
Q

acute post infectious glomerulonephritis features

A

3-7y/o

usually group A strep - beta haemolytic

throat 7-10 days after infection, skin 2-4wks

41
Q

pathogenesis in PIGN

A
  1. nephrogenic antigens on strep
  2. bind specific sites in glomerulus, antibodies bind Ag forming circulating complexes → deposits in the kidney
  3. humeral and cellular immune response, activates alternative complement pathway
  4. AKI
42
Q

prognosis of acute PIGN

A

self limiting

generally improves 6wks after diagnosis - haematuria and hypertension resolves, normalisation of C3 6-8wks after

not recurrent

43
Q

making diagnosis of acute PIGN

A

bacterial culture

+ve AOT

low C3

remeber DDx e.g. lupus, MPGN, IgA nephropathy

44
Q

treatment of PIGN

A

abx

support renal function - electrolyte, acid-base

overload/HT - diuretics

45
Q

how common is IgA nephropathy

A

most common glomerulonephritis

46
Q

features of IgA nephropathy

A

1-2 days after URTI

usually older children and adults

  • recurrent macroscopic haematuria
  • +/- chronic microscopic haematuria
  • varying degree of proteinuria

clinical diagnosis

47
Q

pathogenesis of IgA nephropathy

A
  1. increased circulating levels of Gd-IgA1
  2. production of anti IgA1 antibodies
  3. immune complexes form in the circulation and in situ
  4. immune complexes in the mesangium cause local immune activation and injury → matrix production, mesangial proliferation, glomerular sclerosis, interstitial fibrosis
48
Q

diagnosis of IgA nephropathy

A

clincial picture

-ve AI workup

normal complement

49
Q

treatment of IgA nephropathy

A

mild disease - ACEi for persistent proteinuria or hypertension

mod-severe - immunosuppression (KDIGO)

50
Q

outcome of IgA nephropathy

A

variable

25% ERSF by 10yrs post diagnosis - ?outcome better in children

51
Q

age of onset of Henoch Schonlein purpura/IgA related vasculitis

A

5-15yrs

52
Q

clinical diagnosis of Henoch Schonlein purpura/IgA related vasculitis

A

mandatory: palpable purpura

one out of:

  • abdo pain
  • renal involvement
  • arthritis or arthralgia
  • biopsy - IgA deposition
53
Q

IgA vasculilits

  • how common
  • what conditions does it overlap with
A

most common childhood vasculitis

small vessel vasculitis

overlaps w/ IgA nephropathy - IgA vasculitis w/ nephritis

54
Q

features of IgA vasculitis

A

1-3 days post trigger

  • viral URTI in 70%
  • streptococcus, drugs

duration of Sx - 4-6wks, ⅓ relapse

nephritis - mesangial cell injury

55
Q

treatment of IgA vasculitis

A

symptomatic - joints, gut

glucocorticoid therapy - not helpful in mild nephritis, may help w/ GI involvement

immunosuppression - trial in mod-severe renal disease

long term - HT and proteinuria screening

56
Q

what is an AKI

A

abrupt loss of kidney function, resulting in the retention of urea and other nitrogenous waste products and in the dysregulation of extracellular volume and electrolytes

57
Q

features of AKI

A

anuira/oliguria <0.5ml/kg/hr

hypertension w/ fluid overload

rapid rise in plasma creatinine

58
Q

features of AKI in paeds

A

serum creatinine >1.5x age specific reference (or previous baseline)

UO <0.5ml/kg for >8hrs

59
Q

interpretation fo AKI warning score

A

AKI 1: measured creatinine >1.5-2x reference creatinine/ULRI

2: measured creatinine 2-3x
3: serum creatinine >3x

60
Q

management of AKI

A

prevention

3M’s:

  • monitor - paediatric early warning scores (BP), UO, weight
  • maintain - good hydration, electrolytes, acid-base
  • minimise - drugs (NSAIDS, diuretics)

causes - pre-renal, renal, post-renal

61
Q

pre-renal cause of AKI

A

perfusion problem

  • intravascular depletion resulting in hypoperfusion of glomerulus
  • decreasing GFR → AKI
  • e.g. gastroenteritis, acute haemorrhage, painful laparonephrectomy syndrome, intravascular depletion, liver disease etc
62
Q

renal causes of AKI

A

glomerular disease - HUS, glomerulonephritis

tubular injury - acute tubular necrosis (consequence of hypoperfusion, drugs)

interstitial nephritis - NSAIDs, AI

63
Q

post-renal causes of AKI

A

obstructive uropathies

e.g. pelvis and ureter obstruction, ureteropelvic junctions, bladder stones/tumours, external pressure e.g. constipation, drugs affecting blood emptying in the pelvic floor

64
Q

what is haemolytic uraemic syndrome

A

haemolysis - packed cell volume <10%, Hb <10g/dl and fragmented erythrocyte on blood film

thrombocytopenia - plt <150x109/L

AKI - serum creatinine > age related range (>97th pc), GFR <80mls/min/1.73m2, proteinuria

65
Q

typical HUS causes

A

typical - post diarrhoea

  • entero-haemorrhagic E coli (EHEC), verotoxin producing E coli (VTEC), shiga toxin (STEC)
  • pneumococcal infection
  • drugs
66
Q

typical HUS causes

A

typical - post diarrhoea

  • entero-haemorrhagic E coli (EHEC), verotoxin producing E coli (VTEC), shiga toxin (STEC)
  • pneumococcal infection
  • drugs
67
Q

risk of developing HUS

A

E coli O157:H7 serotype

period of risk of HUS

  • up to 14 days after onset of diarrhoea
  • 15% develop HUS

bloody diarrhoea is a medical emergency in children

68
Q

triad of symptoms in HUS

A

microangiopathic haemolytic anaemia

thrombocytopenia

AKI/acute renal failure

69
Q

consequences of HUS

A
70
Q

management of bloody diarrhoea and HUS - volume expansion

A

prevention of oliguric HUS = intravascular volume expansion w/ normal saline

71
Q

management of HUS - 3 M’s

A

monitor:

  • 5 kidney functions
    • fluid balance - hypertension
    • electrolytes
    • acidosis
    • waste
    • hormones - hypertension

maintain:

  • IV normal saline and fluid
  • renal replacement therapy

minimise:

  • no abx/NSAIDs
72
Q

monitoring for LT consequences of AKI

A

BP

proteinuria

evolution to CKD

73
Q

causes of chronic kidney disease in children

A
  • congenital anomalies of the kidney and urinary tract - 55% of CKD
    • reflux nephropathy, dysplasia, obstructive uropathy
  • hereditary conditions - 17%
    • cystic kidney disease, cystinosis
  • glomerulonephritis - 10%
  • remember syndromic causes - CAKUT may not be isolated
    • turner, trisomy 21, branchio-oto-renal, prune belly syndrome
74
Q

stages of CKD

A
  • normal GFR: 90-120
  • CKD 2: 60-89
  • CKD 3a: 45-59, b: 30-44
  • CKD 4: 15-29
  • CKD 5: ESRF
75
Q

presentation of CKD

A

symptoms variable depending on which function affected

  • waste handling: increased urea → decreased appetite, affects cardiac function, affects neuro function → seizures
  • water handling: poly/oligouria
  • salt balance: hyponatraemia → affects growth, hyperkalaemia → affects cardiac health
  • acid-base control → poor growth
  • endocrine: red cells → anaemia, BP → hypertensive (high renin drive) OR hypotensive (very polyuric), bone health → metabolic bone disease
  • bladder dysfunction
76
Q

UTI in children - definition

A

clinical signs

AND

  • bacterial culture from MSSU
  • any growth on suprapubic aspiration or catheter
77
Q

presenting signs and symptoms in neonates w/ UTI - most to least common (systemic symptoms in bold)

A
  • fever, vomiting, lethargy, irritability
  • poor feeding, FTT
  • abdo pain, jaundice, haematuria, offensive urine

any child can present w/ septic shock 2y to UTI (more common in infants)

78
Q

presenting signs and symptoms in pre-verbal children w/ UTI - - most to least common (systemic symptoms in bold)

A
  • fever
  • abdo or abdo/loin tenderness, vomiting, poor feeding
  • lethargy, irritability, haematuria, offensive urine, FTT

any child can present w/ septic shock 2y to UTI (more common in infants)

79
Q

presenting signs and symptoms in verbal children w/ UTI - most to least common (systemic symptoms in bold)

A
  • frequency, dysuria
  • dysfunctional voiding, changes to continence, abdo/loin pain or tenderness
  • fever, malaise, vomiting, haematuria, offensive urine, cloudy urine

any child can present w/ septic shock 2y to UTI (more common in infants)

80
Q

obtaining urine specimens

A
  • difficult in pre-toilet trained children
  • clean catch or MSSU is gold standard
  • ? collection pads and urine bags
  • sick infants - catheter samples or suprapubic aspiration
  • if acutely unwell, do not delay treatment
81
Q

diagnosing UTI in children

A

suggestive tests:

  • dipstix
    • leucocyte esterase activity, nitrites
    • unreliable <2y/o
  • microscopy
    • pyruria, bacteria

culture >105 colony forming units/ml (gram -ve bacteria - e coli)

82
Q

why are UTI’s worrying in vesico-ureteric reflux

A

may cause a kidney injury

  • not all children w/ reflux get scarring
83
Q

grades of vesico-ureteric reflux

A

higher grade = more likely to cause/have an associated renal injury

  1. reflux partially up ureter
  2. all the way up into the calyces but without distending
  3. ureteric distension
  4. up into fornices and sharp angle in relation to papillae
  5. significant tortuosite of the ureters, loss of papillae indentation into the calyces
84
Q

what to look out for in UTI

A
  • screening for children at risk of progressive scarring
    • reflux nephropathy
    • capture those w/ renal dysplasia
  • urological abnormalities
  • unstable bladder - voiding dysfunction
85
Q

when to investigate a UTI in children

A
  • all children w/ first time UTI
  • upper tract symptoms
  • younger
  • recurrent
86
Q

investigating a UTI

A

US - structure (within 6wks)

DMSA (isotope scan) - scarring/function

micturating cysto-urethrogram MAG 3 scan - dynamic (gold standard for looking for reflux)

87
Q

treatment of UTIs

A

lower tract - 3 days oral abx

upper tract/pyelonephritis - 7-10 days abx (oral if systemically well), possible role of prophylaxis but controversial

prevention - fluids, hygiene, avoiding constipation

manage voiding dysfunction

88
Q

when is prophylaxis used in UTI treatment

A

abnormal urinary tract:

  • grade 3 or above VUR
  • obstructive abnormalities e.g. PUJ obstruction, posterior urethral valves
  • abnormal bladder
89
Q

factors affecting progression of CKD

A
  • late referral
  • hypertension
  • proteinuria
  • high intake of protein, phosphate and salt
  • bone health - PTH, phosphate, vit D
  • acidosis
  • recurrent UTI
90
Q

blood pressure in children

A

gold standard - sphymanomometer

  • doppler US in children <5
  • oscillometry good for MAP

technique is important - children move about and cuff can deflate, never use cuff too small (bladder needs to cover at leat 80% of arm circumference)

  • white coat HT - 24hr ABPM
91
Q

factors affecting BP

A

sex

age

height specific

92
Q

hypertension in children - definition

A

3 occasions, ≥95th percentile

borderline ≥90 but <95th PC

93
Q

management of CKD

A

depends on which function affected

  • modify protein intake
  • fluid restriction/free access if oliguric/polyuric
  • K restriction in ESRF, Na supplementation if hyponatraemic
  • phosphate reduction in diet or binders
  • bicarb replacement
  • EPO for anaemia
  • control BP - ACEi
  • monitor bladder function e.g. ocybutynin to relax bladder function
94
Q

how does metabolic bone disease occur with CKD

A

damaged kidneys → can’t excrete phosphate → phosphate elevates in blood

phosphate = potent driver for PTH

damaged kidneys can’t activate vit D3 into active form → less calcium absorbed from gut into blood and calcium is also lost from kidneys → hypocalcaemia → drives PTH

95
Q

management of metabolic bone disease

A

low phosphate diet

phosphate binders

active vit D

if ongoing poor growth - growth hormone

96
Q

cardiovascular risks in renal patients

A

accelerated atherosclerosis

  • traditional risk factors

PLUS

  • anaemia/metabolic bone disease (PTH)
97
Q

sdf

A

Y3 paediatrics (45 decks)

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