Henoch Schonein Purpura

Question 1

What kind of disease is HSP

Answer 1

an IgA mediated vasculitis (small vessel vasculitis)

Question 2

Explain the aetiology of HSP

Answer 2

URTI > release of IgA (as lungs are mucosal surface) > IgA cross reacts with BV wall via molecular mimickry > immune complex formation

Immune complex travels to different parts of thee body to cause sx

Question 3

What is the average age for HSP

Answer 3

10 years

Question 4

What are features of an HSP rash

Answer 4

Purpuric
PALPABLE (due to swelling from immune complex deposition)
symmetrical distribution over buttocks and extensor surfaces of legs

Question 5

outline key sx of HSP

Answer 5

PAGAP

Purpuric palpable rash 
Abdominal pain, haematochezia, melaenia, haematemesis 
Glomerulonephritis > haematuria 
Arthalgia 
Periarticular oedema

Question 6

Explain GI sx of HSP

Answer 6

Abdominal pain, haematochezia, melaenia, haematemesis

Question 7

How long after URTI does HSP occur

Answer 7

1-2 weeks

Question 8

what investigations should you get first line for HSP

Answer 8

FBC, clotting , renal function, urine dipstick

IgA levels

Question 9

How do you manage HSP

Answer 9

Most cases will resolve spontaneously within 4 weeks

• symptomatic relief (paracet, ibuprofen) for joint pain
• PO/IV corticosteroids if severe
• consider renal transplant if end stage disease

Question 10

what aree complications of HSP

Answer 10

Intussception 
AKI 
Arthritis, arthalgia
Pancreatitis 
Testicular swelling

Question 11

How do you manage phenylalanine diagnosed in neonate

Answer 11

Life long low phenylalanine diet + amino acid supplements

Question 12

what are some complications of PKU

Answer 12

epilepsy
microcephaly
eczema

Question 13

what are possible anatomical lead points for causing insussception

Answer 13

Iliocaecal valve

may have pathological cause e.g. Meckels diverticulum, polyp, lymph node, swollen appendix

Question 14

what will X ray show in intussusception

Answer 14

distended small bowel

no gas in distal colon and rectum

Question 15

What three Ix can you get for intussusception

Answer 15

X ray
USS
Air contrast enema

Question 16

when is rectal air insufflation contraindicated in

Answer 16

ischaemic bowel
peritonitis
hypovolaemic shock
perforation

Question 17

summarise intussusception sx

Answer 17

Paroxysmal, severe colicky pain 
refusing feeds
vomiting 
redcurrant jelly stoool 
sausage shaped mass in RUQ

Question 18

What conditions is intussusception associated with and why

Answer 18

• HSP - intestinal wall haematoma
• CF - hypertrophied mucosal glands
• lymphoma - enlarged mesenteric lymph nodes

Question 19

How do you manage HSP

Answer 19

Supportively - most cases are mild and will resolve spontaneously within 4 weeks

Joint pain - paracteamol/ibuprofen
Scrotal involevemnt / severe oedemaa: oral pred

Question 20

What can you give if HSP and declining renal function?

Answer 20

IV cotritosteroids

Question 21

How long do you follow up HSP for

Answer 21

1 year from episode

weekly follow up with hospital / GP for BP, urine sample

Question 22

what is the most likely cause of macroscopic haematuria in children

Answer 22

IgA nephropathy