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4th Year > Paeds Haem/Oncology > Flashcards

Paeds Haem/Oncology Flashcards

(111 cards)

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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
What are the 3 indications for exchange transfusion?
Acute chest syndrome Stroke Priapism
26
What is acute chest syndrome?
CXR shows bilateral lower consolidation in lungs
27
What is the management of acute chest syndrome?
CPAP Exchange transfusion Abx
28
When would a bone marrow transplant be offered in SCD?
If very severe crises eg. stroke | 90% cure, 5% fatal
29
What is the life expectancy of SCD?
50% die <40yrs | 3% childhood mortality - generally infection
30
When is SCD tested for?
Heel prick test | Allows early prophylactic Abx
31
What is HbSC?
A less severe version of SCD, have half HbS, half HbC
32
What are HbSC patients at risk of?
Proliferative retinopathy - periodic eye checks needed | Osteonecrosis of hips and shoulders in teens
33
What is beta-thalassaemia major?
No HbA present - need bone marrow transplant if possible
34
What is beta-thalassaemia intermedia?
Small amount of HbA and large amount of HbF
35
What is the management of beta-thalassaemia?
Monthly blood transfusions from 3-6 months otherwise fatal
36
What is the potential side effect of having monthly blood transfusions?
Chronic iron overload | Need chelation from 2-3yrs
37
How do you chelate chronic iron overload?
Subcut desferrioxamine | Oral desferasirox
38
Which factors is the PT relevant for?
Factors 2, 5, 7, 10
39
Which factors is the APTT relevant for?
Factors 2, 5, 8, 9, 10, 11, 12
40
What does the thrombin time indicate?
Fibrinogen deficieny
41
What clotting time in relevant for Haemophilias?
APTT
42
What inheritance is Haemophilia?
X-linked | 1/3 is sporadic
43
What factor is deficient in Haemophilia A?
Factor 8
44
What factor is deficient in Haemophilia B?
Factor 9
45
What factors are low in neonates?
All but factor 8 and fibrinogen
46
What %age of factor 8/9 corresponds with severe, moderate and mild disease?
``` Severe = <1% - spontaneous muscle/joint bleeds Moderate = 1-5% - bleed after minor trauma Mild = >5% - bleed after major trauma ```
47
When does Haemophilia present?
About 1yr, when starting to walk - bruise easily 40% neonatally - IC haemorrhage, bleeding post-circumcision, or continued oozing from heel prick test
48
What is the ideal management of Haemophilia A and B?
Recombinant factors 8 or 9 | Aim to raise to 30% of normal to prevent joint/muscle bleeds
49
What factor levels are required for surgery?
100% and 50% maintain for 2 weeks following | Need 12hrly/continuous factor infusion
50
What 3 'medications' need to be avoided in Haemophilia?
All IM injections Aspirin NSAIDs
51
By when is home management encouraged in Heamophilia?
2-3yrs | Child takes over by 7-8yrs
52
What does severe Haemophilia A require?
Prophylactic factor 8 2-3 times per week | Need to keep above 2%
53
Why can desmopressin be used to treat mild Haemophilia A?
Desmopressin increases F8:C and vWF production | Ineffective for Haemophilia B
54
What is a potential complication of have recombinant factors 8/9?
5-20% develop antibodies | Immunosuppression may help
55
What chromosome is relevant in vWD?
Chromosome 12
56
What does vWF do?
Allows platelet adhesion to endothelium | Acts as factor 8 carrier protein - prevents factor 8 clearance
57
What is the inheritance of vWD?
Autosomal dominant
58
What is the severity of vWD?
Generally mild, presents in teens
59
What are the symptoms of vWD?
Easy bruising Prolonged bleeding Epistaxis Menorrhagia Don't often get soft tissue bleeds or haematomas
60
What is the management of vWD?
DDAVP (Desmopressin)
61
Why do you need to be cautious about using desmopressin in children <1yr old?
Can cause water retention and hyponatraemia -> seizures
62
What is the management of severe vWD?
Plasma-derived Factor 8 | DDAVP is inadequate
63
What is the most common type of leukaemia?
ALL
64
When does ALL peak?
2-5 years old
65
What a the 3 main features of ALL?
Anaemia Neutropenia Thrombocytopenia
66
What may be found on examination of someone with ALL?
Bruises Hepatosplenomegaly Enlarged LNs
67
What type of cells may be seen on a blood film in ALL?
Blast cells
68
What is required for a diagnosis of ALL?
Bone marrow biopsy
69
What other investigations (not bloods) are useful in ALL?
LP - checks if blast cells in CSF | CXR - checks for mediastinal LNs
70
What 3 things suggest a worse prognosis?
Age <1 or >10yrs WCC >50 Blast cells in marrow
71
What is the management of ALL?
Chemotherapy Blood and platelet transfusion Allopurinol and hydration protects kidneys
72
What is the chemotherapy regime for ALL?
Induction of remission Intensification to consolidate remission - includes intrathecal chemo to prevent CNS relapse Continuation - 3yrs of moderate chemo
73
What must you give alongside chemo to prevent Pneumocystis in ALL?
Clotrimazole prophylaxis
74
When does Hodgkin and NHL present?
``` HL = teens NHL = children ```
75
What is lymphoma?
Malignancy of immune cells
76
How does Hodgkin lymphoma present?
Painless firm lymphadenopathy for a long time - may cause airway obstruction B-symptoms uncommon
77
What virus is HL associated with?
EBV - 50% of cases
78
What type of cells may be seen on a blood film of HL?
Reed-Sternberg cells
79
What is the management of HL?
Chemo-radiotherapy | 80% cure rate
80
What is the presentation of NHL?
Mediastinal mass if T-cell disease - may cause SVCO Enlarged LNs if B-cell disease Abdo pain if causing intestinal obstruction
81
What is the management of NHL?
Multiagent chemo | 80% cure rate
82
What is Burkitt's Lymphoma?
Type of NHL | c-myc gene
83
What are the 3 variants of Burkitt's Lymphoma?
Endemic variant Sporadic variant Immunodeficiency variant
84
What is the endemic variant?
Common in Africa Almost all have EBV Involves facial and jaw bones
85
What is a neuroblastoma?
Tumour from neural crest tissue in adrenal medulla and sympathetic nervous system
86
When do neuroblastoma occur?
>5yrs | Occasionally young infants - regress spontaneously
87
What are the symptoms of a neuroblastoma?
Large abdominal mass from adrenal glands, often crosses midline and involves major vessels Mets -> bone pain, BM suppression, weight loss
88
What 3 investigations should be performed for neuroblastoma?
Urinary catecholamine metabolites (VMA, VHA) - increased BM biopsy MIBG scan for mets
89
Why is the prognosis often poor for neuroblastoma?
Often present >1yr with mets
90
What is the cure rate for neuroblastoma?
40%
91
What is the management of neuroblastoma?
Single lesion = surgery | Mets = high-dose chemo with stem cell rescue
92
When do Wilm's tumour present?
80% <5yrs
93
What is the usual presentation of Wilm's tumour?
Large abdominal mass found incidentally | Occasionally haematuria
94
What investigations should you perform for Wilm's tumour?
USS +/- CT/MRI | Shows intrinsic renal mass
95
What is the management of Wilm's tumour?
Chemo followed by delayed nephrectomy
96
What is the cure rate for Wilm's tumour?
80% | 60% if mets
97
What is the most common paediatric brain tumour?
Astrocytoma, 40%
98
What is a particularly aggressive form of astrocytoma?
Glioblastoma Multiforme
99
Where would a medulloblastoma be found?
In the midline of the posterior fossa
100
Why do 20% of patients with medulloblastoma have spinal mets?
Spreads through CSF
101
Where would a ependymoma be found?
Posterior fossa - similar to medulloblastoma
102
How common are brainstem gliomas?
4%, very poor prognosis
103
Where are craniopharyngomas found?
Suprasellar region | Grow from remnant of Rathke pouch
104
What visual disturbance can craniopharyngomas cause?
Bitemporal hetronymous hemianopia
105
Where are astrocytomas often found?
Frontal lobe
106
14yr old with aggressive behaviour, seizures and headache
Astrocytoma | <30% survival
107
10yr old with headache, vomiting, poor growth, visual changes and diabetes insipidus
Craniopharyngoma | Good survival rate but long-term vision and pituitary issues
108
3yr old with morning vomiting, unsteady on feet and new squint
Medulloblastoma | 50% 5yr survival
109
4yr old refusing to walk with squint, facial asymmetry and drooling
Brainstem glioma | <10% survival - palliative treatment
110
What investigations do you need for brain tumours?
CT head then MRI | LP - contraindicated in increase ICP
111
What is the management of brain tumours?
Surgery - reduce hydrocephalus and attempt maximal resection, not with brainstem tumours RTx/chemo depends on tumour type

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