Paediatric Nephrology Flashcards
What are the five main functions of the kidney?
- Waste handling
- Water handling
- Salt balance
- Acid base control
- Endocrine (erythropoeitin / RAAS / PTH)
What are two of the main presenting symptoms in nephrology?
Haematuria (indicative of nephritic syndrome)
Proteinuria (indicative of nephrotic syndrome)
What is proteinuria a sign of?
Glomerular injury
Which glomerulopathies tend to affect the epithelial cells (podocytes) of the glomerulus? The basement membrane? The Endothelial cells? The mesangial cells?
Podocyte - minimal change disease
Basement membrane - post infective glomerulonephritis (PIGN)
Endothelial cells - PIGN, haemolytic uraemic syndrome (HUS)
Mesangial cells - HSP / IgA nephropathy
What are the different methods of detecting and quantifying protein in the urine?
Dip stick - (>3+ abnormal) measures concentration
Protein : Creatinine ratio - early morning urine is best (normal < 20, nephrotic >250)
24hr urine collection (gold standard, not that practical)
Descrube the pathophysiology of nephrotic syndrome?
Nephrotic syndrome is characterized by injury to the glomeruli which leads to excessive proteinuria
This causes hypoalbuminaemia
This leads to significant oedema
How does nephrotic syndrome tend to present?
Significant oedema (pitting, periorbital, ascites, small pleural effusions)
Can be hyper, hypo or normo tensive
Frothy urine
Inflated weight
Microscopic haematuria & low urine Na also common
Investigations for suspected nephrotic syndrome?
Urine protein, urine Na
Protein creatinine ratio (urine)
Bloods: albumin, creatinine
What is the most common kidney pathology in children? What clinical features would be expected?
Minimal change disease
- Normal blood pressure
- No frank haematuria
- normal renal function
How is minimal change disease treated in children? What are some possible side effects of treatment?
Prednisolone for 8 weeks (anti-inflammatory)
Personality change
Susceptibility to infection
Hypertension
Increased acid production (GI)
What is one characteristic of MCD with regards to treatment that helps separate it from other glomerulonephropathies?
MCD tends to respond to steroid treatment
What is the prognosis for MCD in children like?
It’s good
80% experience long term remission, others require long time immunosuppression
What causes MCD?
Lympocytes (Ts and Bs) attack podocytes of the glomerulus
What is the most common acquired cause of steroid resistant nephrotic syndrome?
Focal segmental glomerulosclerosis
Characterized by podocyte loss and progressive inflammation and sclerosis
What is the course of action if haematuria is detected in a child?
Frank haematuria - always investigate
Microscopic haematuria - positive 3 times and then investigate, or positive once with accompanying proteinuria
What are some possible causes of haematuria?
Glomerulonephritis Malignancies - sarcomas UTI Stones Trauma Urethritis
What component of the glomerulus is usually injured in the presence of frank haematuria?
Endothelial cell
- Post infective glomerulonephritis
- Haemolytic uraemic syndrome
- Membranoproliferative Glomerulonephritis
- Lupus
- ANCA vasculitis
What investigations may be done to check for post infective glomerulonephritis?
ASOT (positive - checks for strep A)
Bacterial culture
Urine dipstick (blood / protein)
Renal USS
Immunology workup (C3 & C4)
What are some symptoms that may be seen in acute post infective glomerulonephritis?
Haematuria (+/- proteinuria)
Hypertension
Oedema
What is the disease course of post infective glomerulonephritis usually like? How is it treated?
Usually self limiting
Treat with antibiotics and support of renal function:
- Electrolytes / acid base
- Diuretic if hypertensive
What is the most common glomerulonephritis? When does it tend to occur? Who does it tend to occur in?
IgA nephropathy
Tends to occur shortly following an URTI
Usually occurs in older children and adults
How does IgA nephropathy tend to present?
Recurrent frank haematuria +/- chronic microscopic haematuria
Varying degrees of proteinuria
What is the gold standard for diagnosis of IgA nephropathy?
Kidney biopsy
- Should be IgA deposits in the mesangial cells
How is IgA nepropathy treated?
Mild disease: ACEi for hypertension and proteinuria
Moderate to severe disease: immunosuppression
What is the cardinal sign of IgA associated vasculitis?
Palpable purpura (blood spots on the skin)
- IgA collects in the blood vessels and causes inflammation and leaking of blood
In addition to purpura, what other symptoms are needed for diagnosis of IgA associated vasculitis?
One of the following 4:
- Abdominal pain
- Renal involvement
- Arthritis or arthralgia
- Skin Biopsy: IgA deposition
What causes IgA vasculitis? What is the disease course like?
Usually occurs 1-3 days post trigger (viral URTI, streptococcus, drugs)
Duration of symptoms is usually 4-6 weeks (1/3 of patients relapse)
can result in mesangial cell injury
How is IgA vasculitis treated?
Glucocorticoid therapy
Immunosuppression
Hypertension and proteinuria screening
What are some of the cardinal signs of acute kidney injury (AKI)?
- Anuria / oliguria (<0.5mL/kg/hr) for 8 hours
- Hypertension with fluid overload
- Rapid rise in plasma creatinine (>1.5x normal level for the childs age)
What causes pre-renal AKI?
Any condition leading to hypoperfusion of the glomerulus
gastroenteritis / acute haemorrhage
What are some causes of intrinsic renal AKI?
Glomerular disease - haemolytic uraemic syndrome, glomerulonephritis
Tubular injury - acute tubular necrosis (as a consequence of hypoperfusion / drugs)
Interstitial nephritis - NSAIDs, autoimmune
What are some causes of post-renal AKI?
Obstructive uropathies
conditions leading to the obstruction of the ureters
What are some of the characteristic findings in haemolytic uraemic syndrome?
Haemolysis (packed cell volume (PCV) less than 30% / Hb level <10)
Thrombocytopenia (platelet count >150)
Raised serum creatinine (>97th percentile)
GFR decreased
Proteinuria
What causes haemolytic uraemic syndrome (HUS)?
Typical HUS:
- Occurs post diarrhoea
- Other causes (pneumococcal infections / drugs)
Atypical HUS:
- genetic condition due to overactive / inappropriate activation of the complement system
What organism tends to cause the bloody diarrhoea that may lead to HUS in children?
E. Coli
- Bloody diarrhoea is a medical emergency in children
What conditions is HUS a syndrome of?
Microangiopathic haemolytic anaemia
Thrombocytopenia
AKI / acute renal failure
What is the best management technique for haemolytic uraemic syndrome?
Intravascular volume expansion
- Monitor kidney function
- Maintain fluid volume: IV saline and fluid**, renal replacement therapy
- Avoid antibiotics / NSAIDS
What is the general rule for managing acute kidney injuries?
3M’s:
- Monitor: 5 kidney functions (fluid balance, electrolytes, acid base, endocrine, waste)
- Maintain: fluid output (IV saline and renal replacement therapy)
- Minimise drugs
How far does renal function have to drop before creatinine becomes abnormal? How does this look in terms of eGRF?
Renal function has to drop around 40%
Normal GFR - 120
Creatinine abnormal at around GFR - 60
Kidney failure at around GFR - 15
How do children with chronic kidney disease tend to present?
Increased urea (loss of apetite & itchiness)
Polyuria (or oliguria…)
Salt balance (hyponatraemia - abnormal growth, hypokalaemia - cardiac symptoms)
Acid base balance (can also affect growth)
Endcrine (anaemic, hypotensive, bad bone health due to PTH)
Bladder dysfunction
How do symptoms expressed due to UTI differ depending on the age of the child?
Neonates - mainly fever, vomiting, lethargy and irratibility (systemic symptoms)
Children - Frequency, dysuria, abdominal pain or tenderness
What nephrological concerns may be raised in a child with a UTI?
Vesicoureteral reflux causing scarring and damage to the kidneys
What investigations may be done on a child experiencing recurrent UTI’s or UTI’s that are affecting the upper urinary tract?
USS (shows kidney structure)
DMSA (isotope scan - scarring & function of kidneys)
Micturating cytso-urethrogram MAG 3 scan (requires cathterization and active micturition - dynamic)
How are UTI’s treated?
Lower tract - 3 days of antibiotics
Upper tract - 7 days of antibiotics
Prophylaxis - fluids, hygeine and avoiding constipation
How does blood pressure change with age and height of the child? What criteria is necessary for a child to be diagnosed with high blood pressure?
It increases
The child needs to have been found to have BP in the upper 95th percentile on at least 3 occasions
In children with high blood pressure what would you prescribe in order to improve their renal outlook?
ACE inhibitor
Maintaining BP in the 50th percentile improves renal outcome
What are some supplementations that may be required if a child is suffering from CKD regarding acid base balance and endocrine function?
Bicarbonate supplementation
Erythropoeitin supplementation
What treatment may be needed to prevent metabolic bone disease in children with CKD?
Low phosphate diets (lowers PTH)
Phosphate binders (to keep phosphate low)
Active vitamin D supplementation (to counteract PTH)
May require growth hormone supplementation if PTH is still too high to allow for normal growth
If a child has suffered from an acute kidney injury what are some important steps to prevent them from developing chronic kidney disease?
Hypertension and proteinuria monitoring
Prevention of UTI’s
Preservation of normal bladder function