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4th Year > Paeds Haem/Oncology > Flashcards

Paeds Haem/Oncology Flashcards

1
Q

What is the most common paed anaemia?

A

Iron deficient anaemia

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2
Q

What is the most common cause of iron deficient anaemia?

A

Inadequate intake

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3
Q

How much iron does a 1yr old need?

A

8mg/day or 1mg/kg/day

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4
Q

How much iron does a child get from breast milk?

A

1.5mg/L

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5
Q

How much iron does a child get from cow’s milk?

A

0.5mg/L

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6
Q

How much iron does a child get from formula milk?

A

5-9mg/L

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7
Q

What are 2 common causes of inadequate intake of iron?

A

Delay in starting mixed feeds past 12 months

Low iron foods or lots of cow’s milk in diet

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8
Q

What vitamin increases iron absorption?

A

Vitamin C

Found in fresh fruit and veg

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9
Q

What decreases the absorption of iron?

A

Tea (tannin) and high fibre foods (phytates)

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10
Q

When do you start getting symptoms with iron deficient anaemia?

A

Hb <60-70

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11
Q

What are the symptoms of iron deficient anaemia?

A

Tiredness, may feed less
Pale - especially tongue and palmar crease
Pica = eating non-food substances eg. soil, chalk, rubber

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12
Q

How is the diagnosis of iron deficient anaemia made?

A

Blood film = microcytic hypochromic

Low serum ferritin

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13
Q

What are the differentials of iron deficient anaemia? (3)

A

Beta thalassaemia trait - generally Asian/Arabic kids
Anaemia of chronic disease eg. kidney
Alpha thalassaemia trait - microcytic and hypochromic but not anaemic, African/Far Eastern kids

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14
Q

What is the management of iron deficient anaemia?

A 
Oral Sytron (doesn't stain teeth like other supplements)
3 months
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15
Q

How much will the Hb increase by while taking iron?

A

10g/L/wk

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16
Q

Why might there be a failure to respond to oral iron?

A

Coeliac

Blood loss eg. Meckel’s diverticulum

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17
Q

Why is a blood transfusion almost never indicated for iron deficient anaemia?

A

Because the decline is gradual so they can tolerate Hb as low as 20g/L (similar to COPD with oxygen)

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18
Q

What inheritance does Sickle Cell have?

A

Autosomal recessive

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19
Q

What is the mutation in SCD?

A

GAG -> GTG in codon 6 of beta-globin gene

Glutamate -> Valine

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20
Q

What is the main determinant of the severity of SCD?

A

The amount of HbF vs HbS
Usually have about 1% HbF, but can be up to 15%
Hydroxycarbamide increases HbF production

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21
Q

What exacerbates HbS polymerisation?

A

Low oxygen
Cold
Dehydration

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22
Q

How much HbS do those with Sickle trait have?

A

About 40%, asymptomatic

Still transmit to offspring

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23
Q

What is the long-term management of SCD? (4)

A

Prophylactic Abx due to functional asplenism
Folic acid supplement due to increased RBC turnover
Avoid cold, dehydration, excessive exercise and undue stress - especially careful after swimming and playing outside
Hydroxycarbamide if prone to crises

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24
Q

What is the management of an acute SCD crisis?

A

Analgesia, fluids, Abx, Oxygen

+/- Exchange transfusion

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25
Q

What are the 3 indications for exchange transfusion?

A

Acute chest syndrome
Stroke
Priapism

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26
Q

What is acute chest syndrome?

A

CXR shows bilateral lower consolidation in lungs

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27
Q

What is the management of acute chest syndrome?

A

CPAP
Exchange transfusion
Abx

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28
Q

When would a bone marrow transplant be offered in SCD?

A

If very severe crises eg. stroke

90% cure, 5% fatal

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29
Q

What is the life expectancy of SCD?

A

50% die <40yrs

3% childhood mortality - generally infection

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30
Q

When is SCD tested for?

A

Heel prick test

Allows early prophylactic Abx

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31
Q

What is HbSC?

A

A less severe version of SCD, have half HbS, half HbC

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32
Q

What are HbSC patients at risk of?

A

Proliferative retinopathy - periodic eye checks needed

Osteonecrosis of hips and shoulders in teens

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33
Q

What is beta-thalassaemia major?

A

No HbA present - need bone marrow transplant if possible

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34
Q

What is beta-thalassaemia intermedia?

A

Small amount of HbA and large amount of HbF

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35
Q

What is the management of beta-thalassaemia?

A

Monthly blood transfusions from 3-6 months otherwise fatal

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36
Q

What is the potential side effect of having monthly blood transfusions?

A

Chronic iron overload

Need chelation from 2-3yrs

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37
Q

How do you chelate chronic iron overload?

A

Subcut desferrioxamine

Oral desferasirox

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38
Q

Which factors is the PT relevant for?

A

Factors 2, 5, 7, 10

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39
Q

Which factors is the APTT relevant for?

A

Factors 2, 5, 8, 9, 10, 11, 12

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40
Q

What does the thrombin time indicate?

A

Fibrinogen deficieny

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41
Q

What clotting time in relevant for Haemophilias?

A

APTT

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42
Q

What inheritance is Haemophilia?

A

X-linked

1/3 is sporadic

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43
Q

What factor is deficient in Haemophilia A?

A

Factor 8

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44
Q

What factor is deficient in Haemophilia B?

A

Factor 9

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45
Q

What factors are low in neonates?

A

All but factor 8 and fibrinogen

46
Q

What %age of factor 8/9 corresponds with severe, moderate and mild disease?

A 
Severe = <1% - spontaneous muscle/joint bleeds
Moderate = 1-5% - bleed after minor trauma
Mild = >5% - bleed after major trauma
47
Q

When does Haemophilia present?

A

About 1yr, when starting to walk - bruise easily

40% neonatally - IC haemorrhage, bleeding post-circumcision, or continued oozing from heel prick test

48
Q

What is the ideal management of Haemophilia A and B?

A

Recombinant factors 8 or 9

Aim to raise to 30% of normal to prevent joint/muscle bleeds

49
Q

What factor levels are required for surgery?

A

100% and 50% maintain for 2 weeks following

Need 12hrly/continuous factor infusion

50
Q

What 3 ‘medications’ need to be avoided in Haemophilia?

A

All IM injections
Aspirin
NSAIDs

51
Q

By when is home management encouraged in Heamophilia?

A

2-3yrs

Child takes over by 7-8yrs

52
Q

What does severe Haemophilia A require?

A

Prophylactic factor 8 2-3 times per week

Need to keep above 2%

53
Q

Why can desmopressin be used to treat mild Haemophilia A?

A

Desmopressin increases F8:C and vWF production

Ineffective for Haemophilia B

54
Q

What is a potential complication of have recombinant factors 8/9?

A

5-20% develop antibodies

Immunosuppression may help

55
Q

What chromosome is relevant in vWD?

A

Chromosome 12

56
Q

What does vWF do?

A

Allows platelet adhesion to endothelium

Acts as factor 8 carrier protein - prevents factor 8 clearance

57
Q

What is the inheritance of vWD?

A

Autosomal dominant

58
Q

What is the severity of vWD?

A

Generally mild, presents in teens

59
Q

What are the symptoms of vWD?

A

Easy bruising
Prolonged bleeding
Epistaxis
Menorrhagia

Don’t often get soft tissue bleeds or haematomas

60
Q

What is the management of vWD?

A

DDAVP (Desmopressin)

61
Q

Why do you need to be cautious about using desmopressin in children <1yr old?

A

Can cause water retention and hyponatraemia -> seizures

62
Q

What is the management of severe vWD?

A

Plasma-derived Factor 8

DDAVP is inadequate

63
Q

What is the most common type of leukaemia?

A

ALL

64
Q

When does ALL peak?

A

2-5 years old

65
Q

What a the 3 main features of ALL?

A

Anaemia
Neutropenia
Thrombocytopenia

66
Q

What may be found on examination of someone with ALL?

A

Bruises
Hepatosplenomegaly
Enlarged LNs

67
Q

What type of cells may be seen on a blood film in ALL?

A

Blast cells

68
Q

What is required for a diagnosis of ALL?

A

Bone marrow biopsy

69
Q

What other investigations (not bloods) are useful in ALL?

A

LP - checks if blast cells in CSF

CXR - checks for mediastinal LNs

70
Q

What 3 things suggest a worse prognosis?

A

Age <1 or >10yrs
WCC >50
Blast cells in marrow

71
Q

What is the management of ALL?

A

Chemotherapy
Blood and platelet transfusion
Allopurinol and hydration protects kidneys

72
Q

What is the chemotherapy regime for ALL?

A

Induction of remission
Intensification to consolidate remission - includes intrathecal chemo to prevent CNS relapse
Continuation - 3yrs of moderate chemo

73
Q

What must you give alongside chemo to prevent Pneumocystis in ALL?

A

Clotrimazole prophylaxis

74
Q

When does Hodgkin and NHL present?

A 
HL = teens
NHL = children
75
Q

What is lymphoma?

A

Malignancy of immune cells

76
Q

How does Hodgkin lymphoma present?

A

Painless firm lymphadenopathy for a long time - may cause airway obstruction
B-symptoms uncommon

77
Q

What virus is HL associated with?

A

EBV - 50% of cases

78
Q

What type of cells may be seen on a blood film of HL?

A

Reed-Sternberg cells

79
Q

What is the management of HL?

A

Chemo-radiotherapy

80% cure rate

80
Q

What is the presentation of NHL?

A

Mediastinal mass if T-cell disease - may cause SVCO
Enlarged LNs if B-cell disease
Abdo pain if causing intestinal obstruction

81
Q

What is the management of NHL?

A

Multiagent chemo

80% cure rate

82
Q

What is Burkitt’s Lymphoma?

A

Type of NHL

c-myc gene

83
Q

What are the 3 variants of Burkitt’s Lymphoma?

A

Endemic variant
Sporadic variant
Immunodeficiency variant

84
Q

What is the endemic variant?

A

Common in Africa
Almost all have EBV
Involves facial and jaw bones

85
Q

What is a neuroblastoma?

A

Tumour from neural crest tissue in adrenal medulla and sympathetic nervous system

86
Q

When do neuroblastoma occur?

A

> 5yrs

Occasionally young infants - regress spontaneously

87
Q

What are the symptoms of a neuroblastoma?

A

Large abdominal mass from adrenal glands, often crosses midline and involves major vessels
Mets -> bone pain, BM suppression, weight loss

88
Q

What 3 investigations should be performed for neuroblastoma?

A

Urinary catecholamine metabolites (VMA, VHA) - increased
BM biopsy
MIBG scan for mets

89
Q

Why is the prognosis often poor for neuroblastoma?

A

Often present >1yr with mets

90
Q

What is the cure rate for neuroblastoma?

A

40%

91
Q

What is the management of neuroblastoma?

A

Single lesion = surgery

Mets = high-dose chemo with stem cell rescue

92
Q

When do Wilm’s tumour present?

A

80% <5yrs

93
Q

What is the usual presentation of Wilm’s tumour?

A

Large abdominal mass found incidentally

Occasionally haematuria

94
Q

What investigations should you perform for Wilm’s tumour?

A

USS +/- CT/MRI

Shows intrinsic renal mass

95
Q

What is the management of Wilm’s tumour?

A

Chemo followed by delayed nephrectomy

96
Q

What is the cure rate for Wilm’s tumour?

A

80%

60% if mets

97
Q

What is the most common paediatric brain tumour?

A

Astrocytoma, 40%

98
Q

What is a particularly aggressive form of astrocytoma?

A

Glioblastoma Multiforme

99
Q

Where would a medulloblastoma be found?

A

In the midline of the posterior fossa

100
Q

Why do 20% of patients with medulloblastoma have spinal mets?

A

Spreads through CSF

101
Q

Where would a ependymoma be found?

A

Posterior fossa - similar to medulloblastoma

102
Q

How common are brainstem gliomas?

A

4%, very poor prognosis

103
Q

Where are craniopharyngomas found?

A

Suprasellar region

Grow from remnant of Rathke pouch

104
Q

What visual disturbance can craniopharyngomas cause?

A

Bitemporal hetronymous hemianopia

105
Q

Where are astrocytomas often found?

A

Frontal lobe

106
Q

14yr old with aggressive behaviour, seizures and headache

A

Astrocytoma

<30% survival

107
Q

10yr old with headache, vomiting, poor growth, visual changes and diabetes insipidus

A

Craniopharyngoma

Good survival rate but long-term vision and pituitary issues

108
Q

3yr old with morning vomiting, unsteady on feet and new squint

A

Medulloblastoma

50% 5yr survival

109
Q

4yr old refusing to walk with squint, facial asymmetry and drooling

A

Brainstem glioma

<10% survival - palliative treatment

110
Q

What investigations do you need for brain tumours?

A

CT head then MRI

LP - contraindicated in increase ICP

111
Q

What is the management of brain tumours?

A

Surgery - reduce hydrocephalus and attempt maximal resection, not with brainstem tumours
RTx/chemo depends on tumour type

4th Year (15 decks)

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