Paediatric GU - HSP and Nephrotic syndrome

Question 1

What is Henoch-Schonlein purpura?

Answer 1

Henoch Schonlein Purpura - IgA mediated autoimmune hypersensitivity vasculitis involving small blood vessels

Question 2

When does Henoch-Schonlein purpura usually present?

Answer 2

Following an infection or vaccination
Winter months
Mostly in boys
75% <10yo - peak 4-6yo

Question 3

How does Henoch-Schonlein purpura present?

Answer 3

Mildly unwell with low grade fever

Abdominal pain and bloody diarrhoea

Rash

Knee and ankle pain, swelling and tenderness

Renal problems - 40%

Question 4

What rash is seen in Henoch-Schonlein purpura?

Answer 4

Symmetrical erythematous macular rash which evolve into raised purpuric rash

Back of legs, buttocks and ulnar side of arms

Question 5

What renal manifestation can Henoch-Schonlein purpura have?

Answer 5

Glomerulonephritis - microscopic haematuria and mild proteinuria

Nephrotic syndrome

Small minority - end stage failure

Question 6

How is Henoch-Schonlein purpura diagnosed?

Answer 6

Clinical diagnosis but lab results expected:

Urinalysis - haematuria and proteinuria
Bloods - raised WCC, ESR and eosinophilia
Serum IgA raised

Can do abdominal USS

Question 7

What differentials are considered for Henoch-Schonlein purpura?

Answer 7

Intussusception
SLE
Thrombocytopenia

Question 8

How is Henoch-Schonlein purpura managed?

Answer 8

Mostly self limiting - supportive treatment

Avoid NSAID’s for pain

Steroids and immunosuppressants can be trialled - inconsistent results

Question 9

What follow up is required for Henoch-Schonlein purpura?

Answer 9

Blood pressure
Urinalysis

Monthly unto 6 months
1 year check renal function after

Question 10

What happens in nephrotic syndrome?

Answer 10

Damage to the basement membrane of the glomerulus leads to protein leakage

Question 11

What triad is classical of nephrotic syndrome?

Answer 11

Proteinuria >3.5g/day
Hypoalbuminaemia <30g/l
Oedema

Question 12

What is the most common primary idiopathic cause of nephrotic syndrome?

Answer 12

Minimal change glomerulonephritis - podocytes damaged

Question 13

What are some secondary causes of nephrotic syndrome?

Answer 13

SLE
HSP
NSAID's
Leukaemia and lymphoma
Alport
Diabetes
HIV

Question 14

How does nephrotic syndrome present?

Answer 14

Facial swelling - periorbital often first
Frothy urine
Generalised symptoms - fatigue, weakness, no appetite
Thrombosis
Leukonychia
Raised JVP

Question 15

How is nephrotic syndrome diagnosed?

Answer 15

Urinalysis - proteinuria
Protein:creatinine ratio
Renal USS

MSU - rule out UTI
FBC, U&E, LFT, coag, CRP, HIV/hep screen, autoimmune screen, fasting glucose, lipids - rule out secondary causes

Question 16

What is the mortality of nephrotic syndrome?

Answer 16

3%

Question 17

How is steroid sensitive nephrotic syndrome managed?

Answer 17

2/3 respond to prednisolone

80% relapse - cyclophosphamide, ciclosporin or mycphenolate

Question 18

How is steroid resistant nephrotic syndrome managed?

Answer 18

Ciclosporin and tacrolimus

Question 19

What other things are important in the management of nephrotic syndrome?

Answer 19

Pneumococcal vaccine
Low salt diet
Furosemide - oedema

Question 20

What complications are associated with nephrotic syndrome?

Answer 20

Infection - immunoglobulin loss in urine

Thromboembolism and hyperlipidaemia - clotting factors and lipids bound to albumin so increased free levels