9. Paediatric nephrology

Question 1

What are the major diseases in paediatric nephrology?

Answer 1

Proteinuria/Haematuria- (glomerular disease, nephrotic syndrome, nephritic syndrome

AKI- heamolytic uraemic syndrome

CKD- developmentla abnomrlaities, repflux nephropahty/UTI

Question 2

What are 5 functions of the kidneys?

Answer 2

Waste handling
Water handling
Salt balance
Acid base control
Endocrine functions (RBC, BP, bone growth)

Question 3

What does proteinuria signify?

Answer 3

Glomerular injury

Question 4

What is nephritic syndrome?

Answer 4

Increasing haematuria

Intravascular overload

Question 5

What is nephrotic syndrome?

Answer 5

Increasing proteinuria
Intravascular depletion
Leads to hypoalbuminaemia
Oedema increasing third space fluid volume as water isn’t pulled into blood vessels

Question 6

What aspects of the glomerulus are affected by acquired glomerulopathy?

Answer 6

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)

Question 7

What are the different ways to measure proteinuria?

Answer 7

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

Question 8

Describe a run of the mill, absolute classic nephrotic case in children.

Answer 8

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

Question 9

What are the typical features of a nephrotic syndrome

Answer 9

age 1-10 years
normal blood pressure
no frank haematuria
normal renal function

Question 10

What are atypical features of nephrotic syndrome

Answer 10

Abnormal renal function, steroid resistance and suggestions of an autoimmune disease are atypical features and warrant a renal biopsy

Question 11

What are the treatments of nephrotic syndrome and what are the side effects?

Answer 11

Prednisolone 8 weeks

personality change
increased susceptibity to infection
hypertension
GI distress
reduced growth/osteoperosis
sleep disturbance

Question 12

What are the different types of steroid resistant nephrotic syndrome

Answer 12

Acquired- focal segmental glomeruloscerosis (FSGS)
Podocyte loss
Progressive inflammation and sclerosis

Congenital- infant presentations
NPHS1- nephrin
NPHS 2- podocin
Podocyte loss

Question 13

How do you identify haematuria

Answer 13

Macroscopic (seen by naked eye) investigate
Microscopic (dipstick adequate) investigate if positive on two or more occasions or if stix positive and microscopy negative

Question 14

What are the causes haematuria?

Answer 14

Systemic clotting disturbances
Renal glomerulonephrtits
Tumour
Wilm's nephroblastoma
Cysts
Malignancies-sarcomas
Stones
UTI's
Trauma
Urethritis

Question 15

What investigations would you do on a child if glomerulonephritis was suspected?

Answer 15

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

Question 16

What are the clinical features of glomerulonephritis?

Answer 16

Haematuria and proteinuria
Reduced GFR
Oliguria
Fluid overload
Raised JVP
Oedma
Hypertension
Worsebing renal failure

Question 17

Describe the pathogensis of post infectious glomerulonephritis?

Answer 17

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

Question 18

How do you diagnose post infectious glomerulonephritis?

Answer 18

Bacterial culture
Positive ASOT (strep antibody test)
Low C3 abnormalities
Remember- differential diagnosis
May not always be post streptococcal

Question 19

How do you treat post infectious glomerulonephritis?

Answer 19

Antibiotics
Support the 5 renal functions (electrolyte/acid base balance)
Overload/hypertension (diuretics)

Question 20

What is Henoch schonlein purpura?

Answer 20

IgA related autoimmune disease
Can affect the blood vessels (vasculitis) or kindeys (glomuerulonephritis)
Presents with purple purpura
Occurs due to URTI or drugs

Question 21

How do you treat IgA vasculitis?

Answer 21

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

Question 22

How do you confirm IgA nephropathy?

Answer 22

Diagnosis through a clinical picture (negative autoimmune workup, normal compliment)
Confirmation biopsy

Question 23

How do you treat IgA nephropathy

Answer 23

Mild disease ACE inhibitors

Moderate to severe- immune suppresion

Question 24

What is meant by an acute kidney injury?

Answer 24

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

Question 25

What are the different classes of acute kidney injury?

Answer 25

AKI 1- Creatinine 1.5-2x reference
AKI 2- measured creatinine 2-3x reference creatinine
AKI 3- serum creatinine> 3x reference creatinine

Question 26

How do you manage AKI’s?

Answer 26

Monitor )peadiatric early warning score (BP, urine output, weight)

Maintain good hydration

Minimise drugs

(3 M’s)

Question 27

What are the three classes of causes of acute kidney injuries?

Answer 27

Pre renal- perfusion problem
Renal- multiple causes
Post renal- obstructive uropathies

Question 28

What are renal causes of an AKI?

Answer 28

Glomerular disease- Heamolytic ureamic syndrome, glomerulonephritis

Tubular injury- acute tubular necrosis
Drugs

Interstitial nephritis- NSAIDS, autoimmune, drugs