Nephrology Flashcards
Paediatric most common cause for proteinuria/ haematuria
Glomerular disease
- Nephrotic syndrome
- nephritic syndrome
Paediatric most common cause for acute kidney injury
Haemolytic uraemic syndrome
Paediatric most common cause for chronic kidney disease
Developmental anomalies (CAKUT) -reflux nephropathy
5 kidney functions
Waste handling (urea and creatinine) Water handling Salt balance (Na, K, Ca, P) Acid base control (bicarbonate) Endocrine (red cells/ blood pressure/ bone health)
What makes up the glomerular filtration barrier
Endothelial cells:
- Fenestrated
- Vulnerable to immune mediated injury
GBM:
- 2 proteins - Type IV collagen (COL4) 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
What makes up the EM glomerular filtration barrier
Fenestrated endothelial cell,
Glomerular basement membrane,
Podocyte with “slit diaphragms”
Clinical indication of glomerulopathy
Blood and protein
What does proteinuria normally signify?
Glomerular injury
Clinical symptoms of nephritic syndrome (PHARAOH)
Proteinuria Haematuria Azotaemia Red blood cell casts Oliguria Hypertension
Intravascular overload
Clinical symptoms of nephrotic syndrome
Proteinuria
Hypoalbuminaemia
Oedema
Hyperlipidaemia
Intravascular depletion
In acquired glomerulopathy what components are affected
Epithelial cells (podocyte)
- Minimal Change Disease
- FSGS
- Lupus
Basement membrane
- Post infectious glomerulonephritis
- Membranous glomeruopathy
- MPGN (Membranoproliferative glomerulonephritis)
Endothelial cell
- PIGN
- HUS
- MPGN
- Lupus
- ANCA vasculitis
Mesangial cell
- Henoch-Schonlein purpura
- IgA nephropathy
- Lupus
Congenital glomerulopathy layers involved
Podocyte cytoskeletal integrity
(congeital nephrotic syndrome)
-Proteins- Podocin, nephrin
Basement membrane proteins
- Alport syndrome (XL)
- Thin basement membrane disease
Endothelial/ microvascular integrity
-Complement regulatory protein
Testing for proteinuria
Dipstix (>2 usually abnormal)
Protein creatinine ratio (nephrotic range >250mg/mmol)
24hr urine collection (nephrotic range >1g/m2/24hrs)
Nephrotic syndrome treatment
Prednisolone 8 weeks
Second line immunosuppression
Who does nephrotic syndrome clinically present?
pale, periorbital oedema, pitting oedema legs, ascites, small pleural effusions, hypotension, frothy urine
Common cause of nephrotic presentation in children?
Minimal change disease,
Focal segmental glomerulosclerosis
Minimal change disease typical features
age 2-5
normal BP
resolving microscopic haematuria
normal renal function
Atypical features of MCD
What would you consider doing if someone presented with these?
Suggestions of autoimmune disease,
abnormal renal function,
steroid resistance
-Consider renal biopsy
Main side effects noticied by parents in children with glucocorticoid treatment
behaviour change,
mood change,
sleep disturbance
Glucocorticoid treatment, what must the dr think about before
hypertension,
pneumococcal vaccination,
antibiotic prophylaxis
what is on the spectrum for idiopathic nephrotic syndrome in childhood
non relapsing, infrequently relapsing, frequently relapsing, steroid dependent, Steroid resistant (Focal segmental glomerulosclerosis)
Pathology behind steroid resistant nephrotic syndrome
Interaction between lymphocytes (t and B cells) and podocytes
Acquired cause of steroid resistant nephrotic syndrome
Focal Segmental Glomeruloscerosis
causes podocyte loss and progressive inflammation and sclerosis
Congenital causes of steroid resistant nephrotic syndrome
NPHS1-nephrin loss
NPHS2- podocin loss
infant presentation
What does persistent haematuria and proteinuria suggest
glomerular disease
Causes of haematuria
systemic:
-clotting disorders
Renal:
- Glomerulonephritis
- Tumour (Wilm’s nephroblastoma)
- Cysts
Malignancies:
-Sarcomas
Stones
UTI
Trauma
Urethritis
What investigations to use in nephritic syndrome and their results
Creatinine (increased) Electrolytes- hyponatraemia hyperkalaemia FBC- anaemia, no haemolysis Albumin (slightly low) UTI (negative)
Presentation of nephritic syndrome
haematuria proteinuria reduced GFR -oliguria -fluid overload (raised JVP and oedema) - Hypertension -Worsening renal failure (=rapidly progressing)
Case:
7 day history of sore throat
+ nephritic syndrome
Typical cause
Acute Post-Infectious Glomerulonephritis
Acute Post-Infectious Glomerulonephritis common cause and from what site?
Group A strep
Beta haemolytic
Site- throat 7-10 days
Skin 2-4 weeks
Investigations for Acute Post-Infectious Glomerulonephritis as cause for nephritic syndrome
Renal USS ASOT Throat swab ANCA Complement levels ?Biopsy
Treatment for Acute Post-Infectious Glomerulonephritis
Antibiotics
Electrolytes
Diuretics
Second most common cause of nepritis in children
Henoch Schonlein Purpura (IgA related vasculitis)
What is Henoch Schonlein Purpura
Non streptococcal post infectious glomerulonephritis
Also known as IgA vasculitis
What needs to be present for clinical diagnosis of Henoch Schonlein Purpura
-Mandatory palpable purpura
-One of 4: (BARA)
Abdominal pain
Renal involvement
Arthritis or arthralgia
Biopsy- IgA deposition
IgA vasculitis triggers and duration of symptoms
1-3 days post trigger presents:
- viral URTI
- streococcus or
- drugs
Duration:
4-6 weeks
1/3 relapse
Clinical presentation of IgA nephropathy
recurrent macroscopic haematuria
+/- Chronic microscopic haematuria
Varying degree of proteinuria
How to diagnosis IgA nephropathy
Autoimmune workup (negative)
Compliement (normal)
Biopsy
Treatment for IgA nephropathy
mild- ACEi
moderate-severe- Immunosuppression
Causes of nephritic syndrome in children
Post infectious glomerulonephritis
IgA nephropathy
Henoch-Schonlein purpura
Causes of nephrotic syndrome in children
FSGS
minimal change
Acute Kidney Injury definition
Abrupt loss of kidney function
Resulting in:
-the retention of urea and other nitrogenous waste products
And
-Dysregulation of extracellular volume and electrolytes
Acute renal failure presentation
Anuria/oliguria (<0.5ml/kg for >8 hours)
Hypertension with fluid overload
Rapid rise in plasma creatinine (>1.5x age specific reference)
Interpretation of AKI warning score
AK1- creatinine >1.5-2x reference
AK2- creatinine 2-3x reference creatinine
AK3- creatinine >3x reference creatinine
AKI management
Prevention 2Ms -Monitor (Paediatric early warning scores (BP), urine output, weight) -Maintain (Good hydration) -Minimise (drugs)
Causes of AKI
prerenal, intrinsic renal and post-renal
Pre-renal:
-Perfusion problem
Intrinsic renal:
- Glomerular diease- HUS, Glomerulonephritis
- Tubular injury- Acute tubular necrosis (consequence of hypoperfusion or drugs)
- Interstitial nephritis- NSAID, autoimmune, drugs
Post-renal:
-Obstructive uropathies
Causes of Haemolytic-uraemic syndrome
Entero-haemorrhaic E.coli
Pneumoccal infection
drugs
Atypical HUS
Period of risk of HUS after Ecoli o157:H7 serotype
up to 14 days after onset of diarrhoea
Triad of Haemolytic-uraemic syndrome
Microangiopathic haemolytic anaemia
Thrombocytopenia
AKI/ Acute renal failure
HUS management
Monitor: -5 kidney functions Maintain: -IV normal saline and fluid Minimise: -No antibiotics/NSAIDs
AKI long term complications
BP (hypertension)
Proteinuria
Evolution to CKD
Chronic kidney disease causes in paediatrics
Congenital anomalies of the kidney and urinary tract
- Reflux nephropathy
- Dysplasia
- Obstructive uropathy
hereditary conditions:
- Cystic kidney disease
- Cystinosis
Glomerulonephritis
What conditions can be associated with CAKUT
turner
trisomy 21
Branchio-oto-renal
Prune belly syndrome
At what GFR do you begin to see symptoms
<60
Presentation of CKD
Waste handling: -Uraemic- loss of appetitie, weight loss, itch Water: -Polyuria Salt balance: -lethargy Endocrine -renal rickets Bladder dysfunction
UTI definition
Clinical signs plus
- Bacteria culture from midstream urine
- Any growths on suprapubic aspiration or catheter
What urine sample techniques to use for different age groups
clean catch urine or midstream urine
sick infants- catheter sample or suprapubic aspiration
what is vesico-ureteric reflux
e flow of urine travels backwards from the bladder, up the ureters
Grades of vesico-ureteric reflux
grade 1: reflux limited to the ureter
grade 2: reflux up to renal pelvis
grade 3: mild dilatation of ureter
grade 4 :tortuous ureter with moderate dilatation
grade 5: tortuous ureter with severe dilatation of ureter and pelvicalyceal system
Who to investigate when it comes to children and UTI
If:
upper tract symptoms
<6 months
Recurrent UTIs
Investigations to conduct if worried about UTIs
Ultrasound
DMSA (isotope scan)
Micturating cysto-urethrogram MAG 3 scan
Treatment for children with UTIs
Lower tract- 3 days oral antibiotics
Upper tract/ pyelonephritis- 7-10 days
Factors affecting progression of CKD
late referral Hypertension Proteinuria High intake of protein, phosphate and salt Bone heath Acidosis Recurrent UTIs
How to measure childs BP
sphigmanomter
oscillomerty
Blood pressure chart children definition of hypertension
> or equal to 95th percentile
Metabolic bone disease treatment principles
low phospate diet
oral phosphate binders
active vitamin D
