Paeds 4B

Question 1

How is neuroblastoma treated?

Answer 1

Localised primaries can be cured by surgery alone
Metastatic disease will require chemotherapy (it may also require stem cell transplantation and radiotherapy)

Question 2

What percentage of patients with Wilm’s tumour are cured?

Answer 2

80%

Question 3

How is retinoblastoma treated?

Answer 3

Chemotherapy to shrink tumours
Laser treatment of the retina (photocoagulation)
Radiotherapy maybe used in more advanced disease

if eye is not salvageable then enucleation
NOTE: 90% cure rate but many will be visually impaired

Question 4

Which antibiotics are used for suspected bacterial meningitis in children?

Answer 4

< 3 months = IV cefotaxime + amoxicillin
> 3 months = IV ceftriaxone

If > 1 month and caused by H. influenzae, give dexamethasone

Question 5

What is the definitive management for slipped upper femoral epiphysis?

Answer 5

Internal fixation across the growth plate

Question 6

Which type of fluid should be given for maintenance requirements in children?

Answer 6

0.9% NaCl + 5% dextrose

Question 7

How are dehydration corrections calculated when administering fluids?

Answer 7

for a 5% dehydrated 20kg child:

Rehydration: %dehydration x weight x 10 = 5 x 20 x 10 = 1000mL. This is the volume that should be given over 48hrs.

Question 8

How should iron deficiency anaemia be treated?

Answer 8

Oral ferrous sulphate 200 mg tablets (2-3/day)

Continue for 3 months after iron deficiency has corrected

Question 9

What advice would you give to someone who is taking iron tablets for iron deficiency anaemia about side-effects?

Answer 9

May experience adverse effects (constipation, diarrhoea, faecal impaction)
Discomfort could be minimised by taking the iron supplement with food

Question 10

How should treatment for iron deficiency anaemia be monitored?

Answer 10

Re-check Hb after 2-4 weeks (expect 20 g/L rise)
FBC every 3 months for 1 year

Question 11

What are the aspects of treating a neonate with hereditary spherocytosis?

Answer 11

Supportive (maybe blood transfusion)
Folic acid supplementation (RBC more fragile than normal hence they break down and these pts are more anaemic than usual so need more folic acid)
Phototherapy/exchange transfusion - due to increased RBC breakdown –> increased risk of pigment gallstones and jaundice

later they may need splenectomy because they are prone to splenomegaly as the spherocytes get trapped in the splene

Question 12

Outline the aspects of treating hereditary spherocytosis in older children and adults.

Answer 12

Supportive (maybe blood transfusion)
Folic acid supplementation
Splenectomy and vaccination regimen for encapsulated bacteria
Cholecystectomy for gallstones (due to bilirubin from increased rbc breakdown)

Question 13

How can complications of sickle cell disease be prevented?

Answer 13

Protection against infection after splenectomy: Immunisation against encapsulated organisms, Daily oral penicillin
To support the increased need for erythropoiesis: Daily folic acid
To reduce risk of painful crisis: Minimise exposure to cold, dehydration, excessive exercise and hypoxia

Question 14

Outline the treatment of acute sickle cell crises.

Answer 14

Oral and IV analgesia
Good hydration
Antibiotics if necessary
Oxygen
Exchange transfusion (for acute chest syndrome, priapism and stroke)

Question 15

Outline the steps in the analgesic ladder.

Answer 15

Step 1: paracetamol
Step 2: NSAIDS or cocodamol (note you shouldnt give codeine to children <12 due to resp depression)
Step 3: morphine (oromorph) But give PRN naloxone

in children, avoid codeine and dihydrocodeine
avoid aspirin due to Reyes

Question 16

Which drug treatment can be used to reduce sickling in sickle cell patients?

Answer 16

Hydroxycarbamide (Hydroxyurea increases HbF in RBC which makes your red blood cells bigger. It helps them stay rounder and more flexible — and makes them less likely to turn into a sickle shape.)

Question 17

How is haemophilia treated?

Answer 17

Factor 8 concentrate (A)
Factor 9 concentrate (B)
Acute bleed - factor concentrates and anti-fibrinolytics eg tranexamic acid

Question 18

Which medications should be avoided in patients with haemophilia and von Willebrand disease?

Answer 18

IM injections
Aspirin
NSAIDs

(IM injections, aspirin and nsaids increase bleeding risk)

Question 19

Which treatment can be used for mild haemophilia A?

Answer 19

Desmopressin (stimulates endogenous release of vWF)

Question 20

What medical treatment can be used for type 1 von Willebrand disease?

Answer 20

Desmopressin
NOTE: risk of hyponatraemia

More severe disease is treated with factor 8 concentrate

Question 21

Describe the usual natural course of ITP.

Answer 21

In 80% it is an acute, benign and self-limiting disease
Resolves spontaneously within 6-8 weeks
Mild/asymptomatic disease can be managed with observation alone

Question 22

How can severe ITP be managed?

Answer 22

IVIG + corticosteroid + platelet transfusions
Antifibrinolytics (e.g. tranexamic acid) may be used

Question 23

How is DIC treated?

Answer 23

Treat underlying cause
Supportive care
Replacement therapy (platelets and clotting factors)
Restoration of physiological coagulation pathways (e.g. using heparins)

Question 24

How is acute osteomyelitis managed?

Answer 24

High-dose IV empirical antibiotics (for 2-4 weeks)
Switch to oral once clinically recovered (continue for 6 weeks)
Immobilise the affected area
Surgical debridement may be necessary
Possible antibiotics used include flucloxacillin and penicillin

Question 25

How would you counsel a patient with Osgood-Schlatter disease?

Answer 25

Stop or reduce sporting activity
Analgesia - paracetamol and NSAIDs
Intermittent ice packs
Protective knee pads
Stretching