Today’s case is not in the ED. This one unfolds on the ward – the morning after the admission. So imagine yourself there – on the Paeds surgical ward.
6 year old boy was admitted last evening after referral from the local GP to the on-call Surg team.
He has a 24 hour history of increasing central abdominal pain. The pain wa initially periumbilical, but overnight it has localized to the lower abdomen – maybe a bit more on the right. Urinalysis was normal
The surgical team have been on their super-early orning rounds and seen the child – they have written in the notes:
“ tender lower abdo, no rebound, guarding, otherwise soft, afebrile. Bloods all normal. PLAN: Med team consult please.? epurients”
OK, now that is Surg -speak for – we aren’t operating on this kid – not a likely appendix. Turf to med, maybe its constipation?
Half way though your ward round the nurse- coordinator calls you to say: “You had better come see this kid – he has just had a bout of melena”
Hmmmm… not sounding like your average “un-appendix”. So you pop in and see him. Mum is looking worried. His obs are normal, his belly is ‘as described’ by the Surg team. When you pull down his jocks for a gander at his bottom you notice his scrotum is a bit red, and on examination his left testis is tender and there is a definite hydrocoele. Hmmmmm…
OK – can you solve this Paediatric puzzle?
What is going on? Well here is the clincher.
2 days later this rash developed
Rash
What are you going to do next?
Congrats to Dr Bek – who as it turns out practices about 500m down the road from me at the local Aboriginal Medical Service. Bek gave the right and most insightful answer. Good call from Maj and Patrick also – honorable commendations. I happen to know Bek was at a recent talk I gave which covered HSP – so technically she did cheat? But I think she already knew before I got to her!
Now what is HSP?
HSP (Henoch-Shonlein purpura) is an IgA-mediated leukocyoclastic vasculitis which produces a syndrome in children as described by the eponymous gents (and Heberden 30 years earlier). They described the quadrad of: purpura, arthralgia, abdominal pain and melena. Of course, they missed the meat – the renal disease. The disease involves a vasculitis of small vessels in the skin, gut, joints, glomeruli etc. It is pretty much the same disease as IgA nephropahy (Berger’s disease) in adults. HSP ends to occur in young children 4 – 10
How to diagnose HSP?
I love HSP as a diagnosis as it is one of those end-of-the-bed diagnoses which can present in a number of ways – a rash, joint pain, belly aches etc. You have to keep it in you thoughts or you might miss it. The diagnosis is basically clinical – unless it is unclear as to the cause of the rash – so you might check for other causes of purpura. As opposed to ITP etc the platelets will be normal or elevated.
How to treat HSP....
Treatment is mainly symptomatic – analgesia and “surgical” management of complications such as intussusception or torsion. GI bleeding, abdo pain and joint pain are usually treated with oral steroids. Does it work? Well – maybe. There are papers in both direction but they are all small and lack the power to say yes or no. A rough summary is that they might make you belly pain get better sooner, make the likelihood of an operation or CT scan lower and just might improve joint symptoms. Now onto the million dollar question… the beans.
What about the renal disease?
The reason HSP is an important problem in the long term is that there is an incidence of chronic renal disease. Usually a nephritic syndrome (though some mixed or nephrotic). And some of these kids will progress to renal failure. Here is the problem – there are studies that show that early steroids, and even cyclophosphamide do not reduce this progression. So we cannot treat the serious complication.
Monitoring includes urinalysis testing, BP monitoring – as there is about a 7% recurrence rate and the risk of renal disease goes up with recurrence and older-age of the kid.
