Paediatric nephrology Flashcards
1
Q
Functions of the kidneys
A
- Waste handling
- Water handling
- Salt balance
- Acid base control
- Endocrine → red cells, blood pressure, bone health
2
Q
Glomerular filtration barrier
A
- Endothelial cells → fenestrated
- Glomerular basement membrane → 2 proteins
- Podocyte → podocin, nephron
- Mesangial cells → support
3
Q
Nephritic vs nephrotic syndrome
A
- Nephritic
- Increasing haematuria
- Intravascular overload
- Nephrotic overload
- Increased proteinuria
- Intravascular depletion
4
Q
Acquired glomerulonephropathy
A
- Minimal change syndrome → podocyte
- Post-infectious glomerulonephritis → Basement membrane and endothelial cell
- Haemolytic-uraemic syndrome → endothelial cell
- IgA nephropathy/ HSP → mesangial cel
- Systemic lupus erythematosis
5
Q
Congenital glomerulonephropathy
A
- Congenital nephrotic syndrome → podocyte cytoskeletal integrity
- Alport syndrome → basement membrane
- Thin basement membrane disease → basement membrane
- Membrano-proliferative glomerulonephritis → complement regulatory proteins
6
Q
Proteinuria: how much is too much
A
- Dipstix → > 3+ abnormal
- Protein creatinine ratio → >250mg/mol
- 24 urine collection (gold standard) → 1g/m2/24 hours
7
Q
Pathophysiology of nephrotic syndrome
A
- Proteinuria
- Hypoalbuminaemia
- Peripheral oedema
8
Q
Clinical features of nephrotic syndrome
A
- Typical
- Age 1-10
- Normal blood pressure
- No frank haematuria
- Normal renal function
- Atypical
- Suggested autoimmune disease
- Abnormal renal function
- Steroid resistance
- Only then consider renal biopsy
9
Q
Management of nephrotic syndrome
A
- PREDNOS → prednisolone in nephrotic syndrome
- 8 weeks
10
Q
Side effects of high dose glucocorticoids
A
- Cushings syndrome
- Personality changes
- Hypertension
- Increased susceptibility to infection
- GI distress/ increased gastric acid
- Growth retardation
- Emotional lability
- Behavioural changes
- Sleep disturbances
11
Q
Steroid resistant nephrotic syndrome
A
- Acquired → focal segmental glomerulosclerosis (FSGS)
- Congenital
- NPHS1 → nephron
- NPHS2 → podocin
12
Q
Haematuria
A
- Macroscopic/ frank
- Micoscopic
- Investigate
- Haemoglobinuria
- associated proteinuria = glomerular disease
13
Q
Causes of haematuria
A
- Lower urinary tract
- Urethritis
- Trauma
- UTI
- Malignancies →
- Upper urinary tract
- Cysts
- Tumours → Wilm’s nephroblastoma
- Glomerulonephritis
- Systemic → clotting disorder
14
Q
Nephritic syndrome
A
- Clinical diagnosis
- Haematuria and proteinuria
- Reduced GFR
- Oliguria
- Fluid overload → oedema and raised JVP
- Hypertension
- Worsening renal failure → rapidly progressive glomerulonephritis
- Intrarenal cause of Acute Kidney Injury (AKI)
15
Q
Acute post-infectious glomerulonephropathy
A
- Between aged 3-5
- Usually group A strept
- Causes beta haemolytic
- Site
- Throat → 7-10 days
- Skin → 2-4 weeks
- Self-limiting
16
Q
Pathogenesis of PIGN
A
- Nephrotgenic antigens on strep
- Binds to specific sites in glomerulus/ formation of antibody-antigen complex deposit in kidneys
- Activation of immune response/ alternative complement pathway
- Acute kidney injury
17
Q
Diagnosis of PIGN
A
- Diagnosis
- Bacterial culture
- Positive ASOT (anti streptolysin O titer)
- Low C3
18
Q
Treatment of PIGN
A
- Antibiotics
- Support renal function → electrolyte/ acid-base balance
- Overload/ hypertension → diuretics
- Outcome → not often recurrent
19
Q
IgA nephropathy
A
- Most common glomerulonephritis
- 1-2 days after URTI
- Older children and adults
20
Q
Differential of Acute post-infectious glomerulonephritis
A
- IgA nephropathy (Berger’s disease)
21
Q
Clinical features of IgA nephropathy
A
- Recurrent macroscopic haematuria
- +/- chronic microscopic haematuria
- Varied proteinuria
22
Q
Pathogenesis of IgA nephropathy
A
- Increased circulating levels of Gd-IgA1
- Production of antibodies in response (IgA or IgG)
- Immune complexes form in kidneys and circulation
- Invasion of immune complexes to mesangium
- Local inflammation and injury
- Glomerunephritis
23
Q
Diagnosis of IgA nephropathy
A
- Negative autoimmune workup
- Normal compliment
- Definitive diagnosis → renal biopsy
*
24
Q
Treatment of IgA nephropathy
A
- Mild → ACEI for proteinuria
- Moderate/ severe → immunosuppression (KDIGO guidelines)
- Outcomes → better in children
25
Q
Henoch Scholein Purpura related to IgA vasculitis
A
- Age 5-15
- Palpable purpura
- 1 of following
- Abdo pain
- Renal involvement
- Arthritis/ arthralgia
- Biopsy → IgA deposits
26
Q
IgA vasculitis
A
- Most common vasculitis of children
- Affects small vessels
- Associated with IgA nephropathy
- 1-2 days post-trigger
- Viral URTI
- Streptococcus
- Drugs
- 4-6 week duration
- Mesangial cell injury → nephritis
27
Q
Treatment of IgA vasculitis
A
- Symptomatic → joints and gut
- Glucocorticoid therapy → only severe and GI related disease
- Immunosuppression → moderate and severe
- Long-term → hypertension and proteinuria screening
28
Q
Definition of Acute Kidney Injury (AKI)
A
- Acute loss of kidney function leading to:
- Retention of urea and other waste products
- Dysregulated extracellular volume
- Electrolyte imbalance
- Anuria/oliguria (<0.5ml/kg/hr for 8 hours)
- Hypertension of fluid overload
- Raised creatinine >1.5 age specific reference
29
Q
AKI warning stages
A
- AKI 1 → 1.5-2x reference creatinine
- AKI 2 → 2-3x reference creatinine
- AKI 3 → >3x reference creatinine
30
Q
Clinical features of AKI
A
- Raised urea and creatinine
- Fluid overload
- Hyperkalaemia
- Hyperphosphataemia
- Hyponatraemia
- Hypocalcaema
- Metabolic acidosis / bicarb loss
- Hypertension
31
Q
Management of AKI
A
- Prevention
- 3 Ms
- Monitor → Paediatric early warning scores, urine output, weight
- Maintain → hydration, electrolytes, acid-base balance
- Minimise → drugs
32
Q
Causes of AKI
A
- Pre-renal
- Perfusion problems
- Renal
- Glomerular disease
- Tubular injury
- Interstital nephritis
- Post-renal
- Obstructive uropathy
33
Q
Haemolytic-uraemic syndrome (HUS)
A
- Packed cell volume >30%
- Haemoglobin level <10g/dl + fragmented erythrocytes on blood film
- Thrombocytopenia
- AKI
- High serum creatinine
- GFR < 80ml/min/1.73m2
- Proteinuria
34
Q
Cause of HUS
A
- Post-diarrhoea
- Entero-haemorrhagic E.coli (EHEC)
- Verotoxin producing
- Shiga toxin producing (STEC)
- Pneumococcal infection
- Drugs
- Atypical HUS
35
Q
Presentation of HUS
A
- Infection by E.coli 0157:H7
- Up to 14 days post diarrhoea
- Abdominal pain
- Fever
- Vomiting
- Bloody diarrhoea
- Resolution/ HUS (15%)
36
Q
HUS triad
A
- Microangiopathic haemolytic anaemia
- Thrombocytopenia
- AKI
37
Q
Management of haemolytic uraemia syndrome
A
- 3 Ms
- Monitor → fluid balance, electrolytes, acidosis, waste, hormones, other organ function
- Maintain → IV saline + fluids, renal replacement therapy
- Minimise → no antibiotics/ NSAIDs
38
Q
Complications of AKI
A
- Evolution to CKD
- Monitor blood pressure and proteinuria
39
Q
Causes of chronic kidney disease in paediatrics
A
- Congenital anomalies of the kidneys and urinary tract (CAKUT)
- 55% of paediatric CKD
- Reflux nephropathy
- Dysplasia
- Obstructive uropathy → e.g. posterior urethral valves
- Hereditary conditions
- 17%
- Cystic kidney disease
- Cystinosis
- Glomerulonephritis → 10%
40
Q
Presentation of CKD
A
- Symptoms variable depending on which function affected
- Waste handling
- Water handling
- Salt balance
- Acid-base control
- Endocrine → red cells/ blood pressure/ bone health
*
41
Q
Obtaining urine specimen in children
A
- Requires normal social cleanliness
- Clean catch urine or midstream urine
- Collection pads, urine bags → not accurate
- Sick infants → catheter or suprapubic aspiration
- Acutely unwell → don’t delay treatment
42
Q
Diagnosis of UTI
A
- Urine dipstix
- Leucocyte esterase activity
- Nitrites
- Unreliable <2 years old
- Microscopy
- pyuria
- Bacturia
- Culture → > (10)5 colony → gram negative bacteria e.coli
43
Q
Why are UTI’s relevant in paediatrics
A
- Vesico-ureteric reflux disease → kidney injury
- Graded 1-5
- Higher grade associated with renal dysplasia
- Combination of UTI, vesico-ureteric reflux and vulnerable kidney → permanent scarring
44
Q
What to look out for
A
- Screening for children at risk of progressive scarring → reflux nephropathy
- Capture those with renal dysplasia
- Urological abnormalities
- Unstable bladder → voiding dysfunction
*
45
Q
Investigations VUR
A
- Ultrasound → structural
- DMSA (isotope scan) → scarring and function
- Micturating cysto-urethrogram (MAG 3 scan) → dynamic
46
Q
Treatment of UTI
A
- Lower tract → 3 days oral antibiotics
- Upper tract/ pyelonephritis
- Antibiotics for 7-10 days
- Oral if systemically well
- Prevention → fluids, hygiene, constipatio
- Manage voiding dysfunction
47
Q
Risk factors of developing CKD
A
- Late referrale
- Hypertension
- Proteinuria
- Diet high in protein, phosphate and salt
- Poor bone heaoth
- PTH
- Phosphate
- Vitamin D
- Acidosis
- Recurrent UTIs
48
Q
Role fo blood pressure control
A
- Gold standard to monitor hypertension → risk factor to CKD
- Sphygmomanometer
- Oscillometry
- Prevent white coat effect → 24 hours ambulatory BP
49
Q
Factors affecting BP
A
- Sex, age, height
50
Q
Criteria for hypertension
A
- 3 separate ocassiona
- Hypertension → >95 percentile
- Borderline → >90 but <95th percentile
51
Q
Management of CKD
A
- Depends of kidney function affected
- Waste handling → reduce protein intake
- Water handling → fluid restriction
- Salt balance → reduce salt
- Acid-base control
- Endocrine
- Bladder dysfunction
51
Q
Management of CKD
A
- Depends of kidney function affected
- Waste handling → reduce protein intake
- Water handling → fluid restriction
- Salt balance → reduce salt
- Acid-base control
- Endocrine
- Bladder dysfunction
52
Q
Metabolic bone disease in CKD
A
- Kidneys function to excrete phosphate (high phosphate → increase in PTH)
- Kidneys activate vitamin D3
- Treatment
- Low phosphate diet
- Phosphate binders
- Active vitamin D
- Growth hormone → if poor growth
53
Q
Cardiovascular risk in CKD
A
- Accelerated atherosclerosis
- Traditional risk factors + anaemia/ metabolic bone disease (PTH)
