9. Paediatric nephrology Flashcards
What are the major diseases in paediatric nephrology?
Proteinuria/Haematuria- (glomerular disease, nephrotic syndrome, nephritic syndrome
AKI- heamolytic uraemic syndrome
CKD- developmentla abnomrlaities, repflux nephropahty/UTI
What are 5 functions of the kidneys?
Waste handling Water handling Salt balance Acid base control Endocrine functions (RBC, BP, bone growth)
What does proteinuria signify?
Glomerular injury
What is nephritic syndrome?
Increasing haematuria
Intravascular overload
What is nephrotic syndrome?
Increasing proteinuria
Intravascular depletion
Leads to hypoalbuminaemia
Oedema increasing third space fluid volume as water isn’t pulled into blood vessels
What aspects of the glomerulus are affected by acquired glomerulopathy?
Common conditions:
Minimal change disease- podocyte (epithelial cell
Post infectious glomerulonephritis (basement membrane)
Heamolytic uraemic syndrome- endothelial cell
IgA nephropathy- mesangial cell (cells that filter the basement membrane)
What are the different ways to measure proteinuria?
Dipstix- measures concentration (>3+ abnormal), has a lot of false positives adn negatives
Protein:creatinine ratio- early mornignurine best, normal range <20mg/mmol. Nephrotic range >250mg/mmol
24 hour urine collection (gold standard)- Nephrotic range >1g/m2/24 hours
Describe a run of the mill, absolute classic nephrotic case in children.
gastroenteritis 10 days prior
3-4 days of a swollen face (worse in the mornings)
One eye closed, swollen legs
No allergies
Inflated weight, pitting oedema, ascites, pleural effusions
Frothy urine
low albumin, normal creatinine on bloods
What are the typical features of a nephrotic syndrome
age 1-10 years
normal blood pressure
no frank haematuria
normal renal function
What are atypical features of nephrotic syndrome
Abnormal renal function, steroid resistance and suggestions of an autoimmune disease are atypical features and warrant a renal biopsy
What are the treatments of nephrotic syndrome and what are the side effects?
Prednisolone 8 weeks
personality change increased susceptibity to infection hypertension GI distress reduced growth/osteoperosis sleep disturbance
What are the different types of steroid resistant nephrotic syndrome
Acquired- focal segmental glomeruloscerosis (FSGS)
Podocyte loss
Progressive inflammation and sclerosis
Congenital- infant presentations
NPHS1- nephrin
NPHS 2- podocin
Podocyte loss
How do you identify haematuria
Macroscopic (seen by naked eye) investigate
Microscopic (dipstick adequate) investigate if positive on two or more occasions or if stix positive and microscopy negative
What are the causes haematuria?
Systemic clotting disturbances Renal glomerulonephrtits Tumour Wilm's nephroblastoma Cysts Malignancies-sarcomas Stones UTI's Trauma Urethritis
What investigations would you do on a child if glomerulonephritis was suspected?
Increased waste accumulation (high creatinine)
Electrolytes looking for hyponatremia and hyperkalaemia
Full blood count looking for anaemia and heamolysis
Exclude UTI with urine culture
protein:creatnine ratio to look for nephrotic syndrome
What are the clinical features of glomerulonephritis?
Haematuria and proteinuria Reduced GFR Oliguria Fluid overload Raised JVP Oedma Hypertension Worsebing renal failure
Describe the pathogensis of post infectious glomerulonephritis?
Group A strep infection
Antigens of the disease binds in the glomerulus,
This leads to a humeral and cellular immune response
This activates an alternative compliment pathway
How do you diagnose post infectious glomerulonephritis?
Bacterial culture Positive ASOT (strep antibody test) Low C3 abnormalities Remember- differential diagnosis May not always be post streptococcal
How do you treat post infectious glomerulonephritis?
Antibiotics
Support the 5 renal functions (electrolyte/acid base balance)
Overload/hypertension (diuretics)
What is Henoch schonlein purpura?
IgA related autoimmune disease
Can affect the blood vessels (vasculitis) or kindeys (glomuerulonephritis)
Presents with purple purpura
Occurs due to URTI or drugs
How do you treat IgA vasculitis?
treat symptoms e.g. joints, guts
Glucocorticoid therapy- not helpful in mild nephritis
May help with gastrointestinal involvement
Imunosuppresion in moderate to severe renal disease
Long term hypertension and proteinuria screening
How do you confirm IgA nephropathy?
Diagnosis through a clinical picture (negative autoimmune workup, normal compliment)
Confirmation biopsy
How do you treat IgA nephropathy
Mild disease ACE inhibitors
Moderate to severe- immune suppresion
What is meant by an acute kidney injury?
Abrupt loss of kidney function, resulting in the retention of urea and other nitrogenous waste products and in the dysregualtion of extracellular volume and electrolytes
What are the different classes of acute kidney injury?
AKI 1- Creatinine 1.5-2x reference
AKI 2- measured creatinine 2-3x reference creatinine
AKI 3- serum creatinine> 3x reference creatinine
How do you manage AKI’s?
Monitor )peadiatric early warning score (BP, urine output, weight)
Maintain good hydration
Minimise drugs
(3 M’s)
What are the three classes of causes of acute kidney injuries?
Pre renal- perfusion problem
Renal- multiple causes
Post renal- obstructive uropathies
What are renal causes of an AKI?
Glomerular disease- Heamolytic ureamic syndrome, glomerulonephritis
Tubular injury- acute tubular necrosis
Drugs
Interstitial nephritis- NSAIDS, autoimmune, drugs
