Paediatric Haem / Onc Conditions Flashcards
What are the common and less common presentations of HSP?
Common - purpuric rash over legs and buttocks - migrating arthlagia and swelling - abdominal pain Less common - HSP nephritis (HT, proteinuria, haematuria) - intussuception - malaena and haematemesis - scrotal swelling
Describe the management of HSP
Basics - analgaesia for joint pain (paracetamol not NSAIDs) - analgaesia for abdo pain (steroids have evidence) Place & Person - Referral to outpatient paeditrician for ongoing observation Definitive management - Education & supportive care Prevention - continue to review for 6 months (including BP and urine dipstick) - steroids are NOT indicated for nephritis prophylaxis
What are the DDx for brusing in children?
‘SHIELD’ Sepsis (meningoccoal) HSP / HUS / Haemophilia ITP Events (Trauma or non-accidental injury) Leukaemia DIC (usually in the setting of severe illness)
How do you diagnose ITP?
An isolated thrombocytpoaenia (platelet count of
What is the management of ITP?
Basics - stop any active bleeding Place and Person - refer to paediatric haematology Investigations and confirm diagnosis - FBE and blood film to confirm isolated thrombocytopaenia - BMAT if unsure / need to rule out leuakaemia Conservative Management - avoid contact sports / high-risk activities - monitor menstural bleeding if post-pubertal female - avoid NSAIDs and 5-ASA - ongoing follow up and FBE Definitive Management’ - steroids (low or high dose) - IVIg
What is the most dangerous complication of ITP?
Intra-cranial haemorrhage Although the risk if
What are the causes of Fe deficiency anaemia in a child?
Poor stores
- Maternal iron defficiency
- Prematurity / low birth weight
Reduced intake
- Haven’t switched to solids at 6 months
- Vegeterian (adolescents)
Reduced Absorption
- Coeliac disease
Increased Loss
- Meckel’s diverticulum
- Cows milk in the first 12 months (causes micro GI bleeds)
- Menstural loss (adolescents)
What are the “next step” investigations for anaemia?
microcytic –> ferritin + Hb electrophoresis
normocytic –> reticulocyte count
macrocytic –> vit B12 / folate
What are the causes of anaemia in a child?
Microcytic
- Fe defficicency
- Thalasseaemia
Normocytic
- With increased retics
- Haemolysis
- Blood loss
- With decreased retics
- transient erythroblastaemia of childhood (caused by parvovirus B19)
- leukaemia
Macrocytic
- B12 / folate defficiency - rare
What questions should you ask in history when presented with a child with anaemia?
Microcytic
- Fe defficicency
- “Diet, on solids, bleeding from anywhere, drinking cows milk before 12 months, abdmonial pain and bloating, prematurity, low birth weight”
- Thalasseaemia
- “FHx of thalassaemia”
Normocytic
- With increased retics
- Haemolysis
- Jaundiced at birth?
- Blood group? Child and maternal?
- Blood loss
- Bleeding from anywhere?
- Haemolysis
With decreased retics
- transient erythroblastaemia of childhood (caused by parvovirus B19)
- unwell with viral ilness?
- Slapped cheeks?
- leukaemia
- Brusing?
- Severe unusally persistent recurring infections?
Macrocytic
- B12 / folate defficiency - rare
What initial investigations should you order for a child with suspected anaemia?
FBE
Ferritin
Blood Film
Reticulocyte count
What is the management of iron defficiency anaemia in the paediatric population?
- Treat underlying cause
- Diet
- Foods rich in iron
- Avoid cows milk until after 6 months
- Encourage orange juice and vitamin C
- Avoid coffee and tea
- Supplementation
- Oral (tablet or drops) or infusion
- Oral will make stools turn very dark, and cause constipation or diarrhoea
- Beware of iron overdose! Keep in locked cupbpard
What would the coagulations studies of someone with ITP show?
The coags would be normal. This is a disorder fo platelets.
What are the different types of ITP?
Acute
- ITP < 12 months
- Occurs in children 2-5 years old
Chronic
- ITP >12 months
- Occurs in children older than 7 and in adults
- Females > males
Recurrent ITP
- Rare
What is the management of ITP
Conservative - watch and wait
Low dose steroids
High dose steroids
IVIg
Splenectomy is a very last line option
What advice would you give to a parent who has a child with ITP?
- Management options
- Conservative - watch and wait
- 75% of children will recover within 4-6%
- No side effects
- Steroids
- Side effects of steroids
- Quicker recovery
- IVIg
- Quicker recovery
- Would have to stay in hospital
- Conservative - watch and wait
- Risk of IVH
- Risk is <<1%, even with no treatment
- Precaitions
- Avoid contact sports
- Take to hospital if suffers trauma, especially to the head, or if symptoms of drowisness, dysarthria, dysphagia
- AVOID ASPRIN AND NSAIDs
30% of patients with haemophilia will have “inhibitors”.
Explain what this means and how the lab tells?
A raised APTT may suggest
- factor defficiency [what you typically think of in haemophilia]
OR
- factor inhibitor [an antibody made against a factor, such as lupus anticoagulant or antibodies formed by haemophilia patients receiving recombinant factors]
How can the lab tell the difference? Mixing studies
What is the treatment of haemophilia?
Haemophilia A
Mild - Moderate
- DDAVP after Challenge Test
- Tranexamic acid
Severe
- Recombinant factor VIII infusions
Haemophilia B
Mild - Moderate
- Tranexamic acid
Severe
- Recombinant factor IX infusions
In what conditions can DDAVP be used? What needs to be done before it’s use?
VWF Type 1 and Factor VIII defficiency (Haemophilia A)
A challenege test (give DDAVP and monitor factor VIII / VWF levels. Also watch for hyponatraemia / BP)
What is the most common malignany of childhood?
And of this, what is the most common type?
Leukaemia
ALL is the most common (80%)
AML is the next common (20%)
What are the symptoms of Leukaemia?
With which symptoms do acute leukaemis tend to present with, and which do chronic leukaemias tend to present with?
Constitutional symptoms
(B symptoms)
- Fever
- Nigh sweats
- LOW
Symptoms of bone marrow infiltration - ACUTE LEUK
- symptoms of anaemia
- severe, persistent, unusual or recurrent infections
- bruising
Symptoms of other ogran infiltration - CHRONI LEUK
- lymphadenopathy
- splenomegaly
- hepatomegaly
What are the causes of bruising in a child?
Sepsis (meningococcal)
HSP / HUS / Haemophilia
ITP
Events (non accidental injury or trauma)
Leukaemia
DIC (usually in the context of severe illness)
What are the typical features of HSP?
HSP typically presents with the triad of:
- Purpuric rash (non-blanching rash usually over lower limbs and buttocks, may be over posterior aspect of elbows)
- Joint pain / swelling (migratory in nature)
- Abdominal pain
May also consist of
- Renal disease: A nephritic syndrome - haematuria, proteinuria, isolated hypertension, renal insufficiency and renal failure (<1%)
- Subcutaenous oedema of the scrotum, hands, feet and sacrum
- Abdominal - intussusception, malaena, haematemasis, spontaneous bowel perforation or pancreatitis
What basic Ix should you perform for HSP?
Bedside Test
- Urine dipstick
- BP
Labratory Tests
- UEC
- Urine MC&S
What is the management of HSP?
Basics
- Paracetamol for pain relief
Place & Person
- Admit for observations and investigations
- Can usually be managed as an out-patient
Ix and confirm diagnosis
- BP
- urine dipstick
- urine MC&S
- UEC
Defitive management
- Supportive care and frequent monitoring (including BP and urine dipstick)
- HSP nephritis (IgA nephropathy) can occur at the time of HSP, or 6 weeks – 6 months later. Continue to screen for this time.
Prognosis
- The use of prednisolone has not been shown to make clinically important improvements in the rate of long-term renal complications – this was recently removed from the RCH guidelines!
What type of renal syndrome can occur in HSP?
Nephritic syndrome
When does neuroblastoma tend to present in children?
<5 years old
When does a retinoblastoma tend to present in children?
<5 years old
What are the common childhood malignancies?
What is the most common type of SOLID tumor in paeds?
brain tumours!
What are the two types of neurfibromatosis and what are they associated with?
- Neurofibromatosis type I, in which the nerve tissue grows tumors (neurofibromas) that may be benign and may cause serious damage by compressing nerves and other tissues.
- Neurofibromatosis type II, in which bilateral acoustic neuromas (tumors of the vestibulocochlear nerve or cranial nerve 8 (CN VIII) also known as schwannoma) develop, often leading to hearing loss.
What are the top two causes of abdominal mass in a child (for Monash exams)?
Neuroblastoma
Wilm’s Tumour (Nephroblastoma)
What is a neuroblastoma?
A cancer which arises from the neural crest cells that precede the sympathetic ANS & can occur in adrenal medulla (most commonly) or abdominal, thoracic and pelvic ganglia.
in what age group does a neuroblastoma occur?
How does it present?
The most common cance of infancy
Arises in children <5yo
Fatigue, loss of apetite, loss of weight, night sweats
mass in the abdominal cavity
sometimes causing constipation
hypertension (from catecholamine secretion or comression of renal artery)
tacchycardia
May have interesting paraneoplastic effects:
- Opsomyoclonus (dancing eyes, dancing feet syndrome)
- Excessive catecholamines: flushing, tachycardia, HTN
- VIP secretion – severe refractory diarrhea with FTT and low K+
Does neurofibromatosis cause an increased risk of neuroblastoma?
NO
The two are unrelated.
Neurofibromatosis is a SYNDROME characterised by skin lesions, and patients have an increased risk of BRAIN TUMOURS.
Neuroblastoma is a cancer arising from the sympathetic peripheral nervous system, and often presents as a mass in the abdominal cavity
Is a neuroblastoma symptomatic at diagnosis?
yes, it often is.
Moreover, at diagnosis 75% of patients with neuroblastoma have metastases
What is the other name for a Wilm’s tumour?
Nephroblastoma
Is a Wilm’s tumor normally symptomatic?
No
What are the symptoms of a Wilm’s tumour?
- Often asymptomatic mass
- May rupture, bleed and cause pain (20%)
- Haematuria and HTN occur in some patients. Can cause an aquired vWD.
