Henoch Schonein Purpura Flashcards
What kind of disease is HSP
an IgA mediated vasculitis (small vessel vasculitis)
Explain the aetiology of HSP
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
What is the average age for HSP
10 years
What are features of an HSP rash
Purpuric
PALPABLE (due to swelling from immune complex deposition)
symmetrical distribution over buttocks and extensor surfaces of legs
outline key sx of HSP
PAGAP
Purpuric palpable rash Abdominal pain, haematochezia, melaenia, haematemesis Glomerulonephritis > haematuria Arthalgia Periarticular oedema
Explain GI sx of HSP
Abdominal pain, haematochezia, melaenia, haematemesis
How long after URTI does HSP occur
1-2 weeks
what investigations should you get first line for HSP
FBC, clotting , renal function, urine dipstick
IgA levels
How do you manage HSP
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
what aree complications of HSP
Intussception AKI Arthritis, arthalgia Pancreatitis Testicular swelling
How do you manage phenylalanine diagnosed in neonate
Life long low phenylalanine diet + amino acid supplements
what are some complications of PKU
epilepsy
microcephaly
eczema
what are possible anatomical lead points for causing insussception
Iliocaecal valve
may have pathological cause e.g. Meckels diverticulum, polyp, lymph node, swollen appendix
what will X ray show in intussusception
distended small bowel
no gas in distal colon and rectum
What three Ix can you get for intussusception
X ray
USS
Air contrast enema
when is rectal air insufflation contraindicated in
ischaemic bowel
peritonitis
hypovolaemic shock
perforation
summarise intussusception sx
Paroxysmal, severe colicky pain refusing feeds vomiting redcurrant jelly stoool sausage shaped mass in RUQ
What conditions is intussusception associated with and why
- HSP - intestinal wall haematoma
- CF - hypertrophied mucosal glands
- lymphoma - enlarged mesenteric lymph nodes
How do you manage HSP
Supportively - most cases are mild and will resolve spontaneously within 4 weeks
Joint pain - paracteamol/ibuprofen
Scrotal involevemnt / severe oedemaa: oral pred
What can you give if HSP and declining renal function?
IV cotritosteroids
How long do you follow up HSP for
1 year from episode
weekly follow up with hospital / GP for BP, urine sample
what is the most likely cause of macroscopic haematuria in children
IgA nephropathy
