Paediatric Nephrology Flashcards
What is the normal GFR for a neonate and a 2yr old?
- Neonate: 20-30ml /min/1.73m2
- 2yrs: equals adult 90-120
What are the five functions of the kidneys?
- Waste handling
- Water handling
- Salt balance
- Acid base control
- Endocrine: red cells/ blood pressure/ bone health
Name the components of the glomerular filtration barrier
- Endothelial cell
- GBM
- Podocyte
- Mesangial cells
What is proteinuria a sign of?
Glomerular injury
What are the features of nephritic syndrome?
- Increasing haematuria
- Intravascular overload
What are the features of nephrotic syndrome?
- Increasing proteinuria
- Intravascular depletion
Name some of the common acquired glomerulopathies and which component they affect
- Minimal change disease: podocytes
- Post infectious glomerulonephritis: basement membrane and endothelial cell
- Haemolytic uraemic syndrome: endothelial cell
- HSP/IgA nephropathy: mesangial cell
Name some of the congenital (rare) glomerulopathies
- Congenital nephrotic syndrome
- Alport syndrome
- Thin basement membrane disease
- Membranoproliferative glomerulonephritis
How can proteinuria be measured and how much is abnormal?
- Dipstix: > 3+
- Protein creatinine ration >250mg/mmol is nephrotic
- 24hr urine collection > 1g/m2/24hrs
What are the features of nephrotic syndrome?
- Nephrotic range proteinuria
- Haematuria (not frank)
- Hypoalbuminaemia
- Oedema
- Low albumin
- Normal creatinine, blood pressure and renal function
What are the atypical features of nephrotic syndrome?
- Suggestion of autoimmune disease
- Abnormal renal function
- Steroid resistance
How can nephrotic syndrome be treated?
Prednisolone for 8 weeks (if typical features)
What are the side effects of high dose glucocorticoids?
- Personality changes
- Hypertension
- Increased in GI acid
- Reduced growth
- Increased susceptibility to infection (think of varicella status, pneumovax and antibiotic prophylaxis)
- Sleep disturbance
What are the causes of steroid resistant nephrotic syndrome
- Acquired: focal segmental glomerulosclerosis
- Congenital: infant presentations, NPHS1/2 and podocyte loss
What are the causes of haematuria?
- Clotting disorders
- Glomerulonephritis
- Tumours
- Cysts
- Stones
- UTI
- Trauma
- Urethritis
How can frank haematuria be investigated?
- Bloods: creatinine, U&Es, FBC and albumin
- Urine: urine culture and protein creatinine ratio
What are the features of nephritic syndrome?
- Haematuria
- Proteinuria
- Oliguria
- Fluid overload: raised JVP and oedema
- Hypertension
- Self limiting
How can post infectious glomerulonephritis be investigated?
- Renal USS
- ASOT
- Throat swab fro group A strep
- C3
- Autoimmune immunology
- Biopsy is not required
How can acute post infectious glomerulonephritis be treated?
- Antibiotics
- Support of renal function (electrolyte and acid base)
- Diuretics (overload/hypertension)
What are the features of IgA related vasculitis (Henoch Schonlein purpura)?
- Non strep post infectious GN (1-3 days after infection)
- Palpable purpura plus one of:
- Abdo pain
- Renal involvement
- Arthritis or arthralgia
- Biopsy
How can IgA vasculitis be treated?
- Symptomatic
- Glucocorticoid (not in mild)
- Immunosuppression
- Long term hypertension and proteinuria screening
How does IgA nephropathy present?
- Older children and adults
- Recurrent macroscopic haematuria +/- chronic microscopic haematuria
- Varying degree of proteinuria
How can IgA nephropathy be diagnosed?
- Clinical picture
- Negative autoimmune workup
- Confirmation biopsy
How can IgA nephropathy be treated?
- Mild: ACE inhibitors
- Moderate to severe: immunosuppression
What is the definition of an acute kidney injury?
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
What are the features of acute renal failure?
- Anuria/oliguria
- Hypertension with fluid overload
- Rapid rise in plasma creatinine
Describe the stages in the AKI warning score
- AKI 1: creatinine > 1.5-2x reference
- AKI 2: creatinine 2-3x reference
- AKI 3: creatinine > 3x reference
How can AKI be prevented?
- Monitor: paediatric early warning scores, urine output and weight
- Maintain good hydration
- Minimise drugs
What are the causes of AKI?
- Perfusion problem
- HUS and GN
- Acute tubular necrosis (hypoperfusion and drugs)
- Interstitial nephritis (NSAIDs, autoimmune and drugs)
- Obstructive uropathies
What are the causes of haemolytic-uraemic syndrome?
- Post diarrhoea
- E coli (entero-haemorrhagic or verotoxin) or shiga toxin
- Other causes: pneumococcal infection and drugs
- Atypical HUS
How does haemolytic uraemic syndrome present?
- Microangiopathic haemolytic anaemia
- Thrombocytopenia
- AKI/Acute renal failure
How can haemolytic uraemic be managed?
- Monitor kidney functions
- IV normal saline and fluids
- Renal replacement therapy
- No antibiotics and NSAIDs
What are the long term consequences of an AKI?
- Blood pressure increase
- Proteinuria monitoring
- Evolution to CKD
Give examples of chronic kidney diseases that can occur in children
- Reflux nephropathy
- Dysplasia
- Obstructive uropathy
- Cystic kidney disease
- Cystinosis
- GN
Which syndromes can be associated with chronic kidney disease?
- Turners
- Trisomy 21
- Branchio-oto-renal
- Prune belly syndrome
How does chronic kidney disease present?
- Loss of appetite
- Weight loss
- Polyuria
- Lethargy
- Reduced effort tolerance
- UTIs
What is the definition of a UTI in children?
- Clinical signs
- Bacteria culture from midstream urine
- Any growth on suprapubic aspiration or catheter
What are the principles of clinical findings of a UTI in children?
- The youngger the age the more systemic symptoms
- The older the age the more lower tract symptoms
How can UTIs be diagnosed in children?
- Dipstix (leukocytes)
- Microscopy: pyuria and bacturia
- Urine culture: E coli
- USS
- DMSA (isotope scan)
- Micturating cysto-urethrogram
What are the potential consequences of UTIs in children?
- Vescico-ureteric reflux
- Scarring
What is the management of UTIs in children?
- Lower tract: 3 days oral antibiotics
- Upper tracts: 7-10 antibiotics, prevention, fluids, hygiene and constipation
- Manage voiding dysfunction
Which factors affect the progression of CKD?
- Late referral
- Hypertension
- Proteinuria
- High intake of protein, phosphate and salt
- Bone health: PTH and phosphate
- Acidosis
- Recurrent UTIs
How can CKD be managed?
- Nutrition/protein intake/ minimise weight loss
- Low potassium diet
- Bicarbonate replacement
- Erythropoetin if anaemic
What are the treatment principles for metabolic bone disease?
- Low phosphate diet
- Oral phosphate binders
- Active vitamin D
- If ongoing poor growth: growth hormone
