arthritis with pain and swelling - knees and ankles
cramping abdominal pain, GI bleed - complication intussusception ileoileal
renal: haematuria, proteinuria in a minority of patients. can be severe. clinical glomerulonephritis or nephrotic syndrome
differential diagnosis of HSP
other cause of abdominal pain
other causes of purpuric or petechial pain eg ITP
how is diagnosis of HSP made?
what are the useful Ix for HSP?
FBC - normal platelet count rules out ITP
renal funciton U&E
US abdo - rule out intussecption
what is Rx of HSP?
symptomatic and supportive
paracetamol or NSAIDs control pain
oral steroids occasional used if severe abdominal pain but ensure intussesception not present
what is prognosis of HSP
majority full recovery in 4-6 weeks
what does long term prognosis of HSP depend on?
presence and severity of NEPHRITIS
why do you have to follow up HSP for prolonged period?
to monitor renal function
what % of pts with HSP progress to end stage renal failure
only 1% pts progress to end stage renal disease
what is the cut off for thrombocytopenia?
platelets < 150 x 10x9
what is the most common cause of thrombocytopenia in children?
ITP - idiopathic thrombocytopenic purpura
what is the cause of ITP?
but immune mediated - anti-platelet antibodies
happens 1-2 weeks after viral infection
as a compensatory mechanism to reduced platelet, what increases in the BM?
what is the peak age of ITP?
2-10 years old
what are the features of ITP?
bleeding from mucosal surfaces eg epistaxis
spleen palpable in some cases
where are the platelets destroyed in ITP?
reticuloendothelial system - mainly spleen
what is a complication of ITP?
but only if long period of severe thrombocytopenia
what is the differential diagnosis of ITP?
Henoch Schonlein Purpura
what Ix are to be done in suspected ITP?
FBC - should not be pancytopenia
Bone marrow aspiration - exclude marrow infiltration - only if doubt about diagnosis (eg if hepatosplenomeg or lymphadenopathy) or starting steroid (as may mask other sinister causes). will see increased megakaryocytic
what is the Rx of most children?
most - disease is acute, benign and self limiting - no therapy needed (even at platelet level <10)
IvIg or steroids
IvIg: cause more rapid rise in platelets, use in severe disease
steroids - only as short course (SE) - always examine BM first to exclude ALL
why are platelet transfusions not effective, when used?
not effective as they will be destroyed
use if life threatening emergency
why is serious bleeding rare?
as platelets function more efficiently
what advice is given to parents in ITP
need 24 hours immediate access to hospital
child avoid trauma and contact sports while plt count v.low
if medical therapy fails what is the next option?
which children (age and sex) have a higher risk of chronic disease?
what is chronic ITP?
low platelets for 6 months after diagnosis
what is treatment for ITP?
avoid contact sports, but encouraged to continue normal activity eg school
24h access to hospital
ITP Support Group
what happens to most children with chronic ITP?
remit within 3 years from onset or stabilise with moderate asymptomatic thrombocytopenia
if ITP in a child becomes chronic, what needs to be screened for?
what is the commonest cause of anaemia in childhood?
how long do term infants have iron reserves for?
how long do preterms have iron reserves for?
by 8 weeks gone as higher rate of growth
what % of iron is absorbed from breast milk?
what % of iron is absorbed from unmodified cows milk?
what are dietary sources of iron?
red meat, liver, kidney, oily fish eg sardines
dark green veg eg spinach, broccoli
what can enhance iron absorption?
what can reduce Iron absorption?
tannin in tea
apart from diet what are other causes of IDA?
what are symptoms of severe anaemia?
what are long term consequences of iron deficiency?
how is diagnosis of anaemia confirmed?
FBC, blood film, iron stores
what is Rx of IDA?
dietary advice: see dietician and inc iron rich food, avoid cows milk, more breast or formula as had added iron
oral iron supplements eg sodium iron edentate
how long is Rx for IDA?
continue for another 3 months AFTER correction of anaemia
to allow replenishment of stores
if there is a failure of response to Rx or recurrence despite adequate intake of iron, what is to be done?
Ix for GI bleed
what causes haemophilia A? and what is inheritance?
factor 8 deficiency
x linked recessive
what are symptoms of haemophilia A?
spontaneous or traumatic bleeding
skin, muscle joints
what is the APTT in haemophilia A?
indicating defect in INTRINSIC pathway
what is Rx of haemophilia A?
replace factor 8
how can mild haemophilia A be treated?
infusion of desmopressin that releases factor 8 from tissue stores
what is the other name of haemophilia B? what is it?
x linked recessive
factor 9 deficiency
what is Rx of haemophilia B?
prothrombin complex concentrate
what are the 2 major roles of von willebrand factor?
carrier protein for F8 - prevent from being broken down