Paediatric Nephrology Flashcards
Breaking it Down
Haematuria/Proteinuria = Glomerular disease
glomerular disease = nephrotic/nephritic syndromes
Acute Kidney injury = Haemolytic uraemic syndrome
Chronic Kidney injury
Developmental anomalies - Reflux nephropathy
the nephron and GFR
Nephron receives 25% of CO/minute
GFR - neonate = 20-30ml/min/1.73m^2
by aged 2 this increases to around 90-120
5 kidney Functions - Do not change in adulthood
Waste - Urea and creatinine
Ion balance - Na/Cl/K
Acid base control - Bicarb
Endocrine - RAAS/PTH/activation of Vit D
Glomerulonephropathy
Blood and Protein in varying volumes will dictate: Clinical presentation and may suggest Dx
However:
damage to one area of GFB affects the other components too
HAEMATURIA = GLOMERULAR INJURY
Acquired glomerulonephroathy
M/C damaged = Epithelial cells (podocytes)
minimal Change, FSGN, Lupus
Basement Membrane - Membranous GN, MPGN, PIGN
Endothelial Cell - PIGN, HUS, MPGN, Lupus
Mesangium - IgA, HP, Lupus
Congenital is more rare
Podocyte Cytoskeleton
Basement membrane proteins
Endothelial/microvascular integrity
Definition of Nephrotic Syndrome
Nephrotic range proteinuria
Hypoalbuminaemia (normal is 36-44g/ - oedema at 25-30)
Oedema ( hypoalbuminaemia –> lack of oncotic pressure and vessels leak)
Typical Presentation of Nephrotic Syndrome
2.5 year old
Gastroenteritis 10 days ago
3-4 day hx of swollen face (worse in mornings) one eye closed in morning, swollen legs
No allergies
Great grandmother had a nephrectomy
Presentation of Nephropathy
Looks well but a bit pale Periorbital oedema, pitting in legs, ascities, small pleural effusions BP Normal or mildly raised FROTHY URINE
Ix for Nephrotic Syndrome
FBC - Albumin Low Creatinine normal
Urine Dip - ++++ protein (24hr urine collection should be done + protein creatinine ratio)
BP
Renal function
Common Causes of Nephrotic Syndrome
Minimal Change GN
Typical features - 2-5yrs, Norm BP, Micro haematuria Normal Renal Function
Atypical features - Suggestions of autoimmune, Abnormal Renal Function, Steroid resistance
ONLY CONSIDER Bx IF ATYPICAL
Mx of nephrotic syndrome
Typically 8weeks prednisolone
S/E Varicella status Pneumococcal Vaccination CUSHINGS BEHAVIOUR
Common Causes of Nephrotic Syndrome
Minimal Change GN
Typical features - 2-5yrs, Norm BP, Micro haematuria Normal Renal Function
Atypical features - Suggestions of autoimmune, Abnormal Renal Function, Steroid resistance
ONLY CONSIDER Bx IF ATYPICAL
Mx of nephrotic syndrome
Typically 8weeks prednisolone
S/E Varicella status Pneumococcal Vaccination CUSHINGS BEHAVIOUR
Why would nephrotic syndrome be steroid resistant
Focal segmental GN - loss of podocytes and progressive inflammation with sclerosis
Congenital presentation also
Microscopic Haematuria Confirmation
If you investigate and there is more than tract on 2 or more separate occasions
(Macroscopic is easier as you will see the blood)
Haemaglobinuria
Stix positive + Microscopy Negative
Persistent haematuria + proteinuria =
Glomerular disease
causes of Microscopic haematuria
Glomerulonephritis: Post infective, IgA/HSP, MPGN, Lupus, ANCA positive vasculitis
UTI/Trauma/Stones
Benign recurrent haematuria (family Hx
Alports syndrome
Thin basement membrane
HUS
Nephrotic Syndrome
MCGN
FSGN
Wilm’s tumour
Sickle cell
Exercise
Clotting abnormalities
Causes of Macroscopic haematuria
Glomerulonephritis: Post infective, MPGN, Lupus, IgA/HSP
UTI
Trauma
Stones
Benign recurrent (FHx) Alports
HUS
Wilm’ tumour
Clotting abnormalities
What is nephritic Syndrome (clinical Diagnosis)
Describes glomerulonephritis
Haematuria and Proteinuria
Reduce GFR - Oliguria, Fluid overload (raised JVP, oedema), Hypertension, Worsening renal failure= Rapidly progressing GN)
Intrarenal Cause of acute kidney injury (AKI)