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Yr 5 - Paeds > Revision - Haem, Hearing Loss > Flashcards

Revision - Haem, Hearing Loss Flashcards

1
Q

What genetic abnormality is seen in 90% of CML cases?

A

Philadelphia chromosome

t(9;22)

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2
Q

What 2 viruses can predispose to leukaemia?

A

EBV & HIV

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3
Q

What may a blood film show in leukaemia?

A

Shows the presence of blast cells.

Note - there may be a false negative if blasts are confined to bone marrow).

Blast cells should normally not be seen in peripheral blood, so this is highly suspicious for leukaemia if seen on microscopy.

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4
Q

Why is G6PD level important in leukaemia?

A

G6PD deficiency should be identified before commencing rasburicase as it can result in a haemolytic crisis.

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5
Q

What is rasburicase?

A

A drug given before and during chemotherapy to treat some types of cancer.

It can help to prevent tumour lysis syndrome.

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6
Q

Why is a CXR relevant in leukaemia?

A

Extremely important to identify early if a MEDIASTINAL MASS is present before the child receives any anaesthetic.

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7
Q

What bone marrow biopsy finding is diagnostic of leukaemia?

A

≥20% blast cells

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8
Q

At the end of induction chemotherapy in leukaemia, what should the blast cell count be for patients to be classed as being in remission?

A

≤5%

Presence of residual disease (i.e. persistent leukaemic cells) indicates the need for more intensive chemotherapy.

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9
Q

What are the 4 stages of chemo for ALL?

A

1) Induction

2) Consolidation and CNS treatment

3) Delayed intensification

4) Maintenance

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10
Q

What is the induction phase of chemo for ALL?

A

What - An intensive phase lasting 4-6 weeks.

Purpose - Aims to destroy all leukaemic blast cells.

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11
Q

To reduce the risk of tumour lysis syndrome in chemo for leukaemia, what can be given prior to induction?

A

Pre-phase treatment with hydration and allopurinol/rasburicase is given prior to chemo.

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12
Q

What is the aim of the consolidation and CNS treatment stage of chemo for ALL?

A

Aim is to maintain remission.

Lumbar puncture with intrathecal methotrexate aims to prevent spread to the CNS.

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13
Q

When can rasburicase be used in prophylactic management of tumour lysis syndrome?

A

If WCC >50

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14
Q

Mechanism of rasburicase vs allopurinol in tumour lysis syndrome?

A

Rasburicase - actively breaks down the uric acid

Allopurinol - prevents uric acid production

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15
Q

What should always be EXCLUDED before giving rasburicase?

A

G6PD deficiency - as it increases the risk of haemolytic crisis.

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16
Q

What is the most common treatment for CML?

A

Tyrosine kinase inhibitors

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17
Q

What is ITP?

A

Involves the development of a purpuric rash in those with low circulating platelets (<100 x 10⁹/L) in the absence of any clear cause.

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18
Q

What type of reaction is seen in ITP?

A

Type II hypersensitivity –> the production of antibodies that target and destroy platelets.

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19
Q

What does ITP usually follow in children?

A

Viral illness

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20
Q

Mx of ITP?

A

1) Normally supportive (will resolve spontaneously within 3 months)

2) Oral prednisolone +/- IVIG

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21
Q

What mutation is present in sickle cell?

A

ingle point mutation in the beta-globin gene on chromosome 11.

This results in amino acid replacement in the beta-globin gene, from gutamic acid to valine.

This results in sickled haemoglobin (HbS).

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22
Q

What are aplastic crises usually precipitated by in sickle cell?

A

Parvovirus B19 infection

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23
Q

There is a marked drop in Hb in both splenic sequestration crisis and aplasic crisis in sickle cell.

How can you tell them apart on a FBC?

A

Splenic sequestration –> rise in reticulocytes

Aplastic crisis –> no rise in reticulocytes

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24
Q

What is recurrent splenic sequestration an indication for?

A

Splenectomy

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25
Q

What investigation is required for diagnosis of sickle cell?

A

Haemoglobin electrophoresis

Will reveal presence of HbS as well as absent or decreased HbA levels.

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26
Q

How are infections prevented in sickle cell?

A

Oral penicillin prophylaxis is recommended until at least age five but is often continued life-long.

Vaccinations: regular childhood vaccinations plus vaccinations against meningococcus, pneumococcus, hepatitis B and influenza.

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27
Q

How is severe anaemia prevented in sickle cell?

A

Folic acid supplementation

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28
Q

What is the mx of thalassaemia?

A

Repeated transfusion

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29
Q

Give 3 complications of thalassaemia major?

A

1) severe microcytic anaemia

2) bone deformities

3) splenomegaly

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30
Q

What can potentially be curative in thalassaemia?

A

Bone marrow transplant

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31
Q

Inheritance of hereditary spherocytosis?

A

Autosomal dominant

32
Q

What is aplastic crisis in hereditary spherocytosis typically preceeded by?

A

Parvovirus infection

33
Q

Inheritance of G6PD deficiency?

A

X-linked recessive (i.e. usually affects males).

34
Q

What may be seen on a blood film in G6PD deficiency?

A

Heinz bodies

35
Q

What are some medications that trigger haemolysis and should be avoided in G6PD deficiency?

A

Primaquine (an antimalarial)

Ciprofloxacin

Nitrofurantoin

Trimethoprim

Sulfonylureas (e.g gliclazide)

Sulfasalazine and other sulphonamide drugs

36
Q

Give 3 causes of congenital hearing loss

A

1) Maternal rubella or CMV infection during pregnancy

2) Congenital deafness

3) Syndromes e.g. Down’s

37
Q

What is the 1st line investigation that is performed when a patient complains of hearing difficulties?

A

Audiogram

38
Q

What conduction is impaired in sensorineural vs conductive hearing loss?

A

Sensorineural –> both air and bone conduction are impaired

Conductive –> only air conduction is impaired

39
Q

What is plotted on the x & y axis of an audiogram?

A

x axis - frequency (Hz)

y axis - volume (dB)

40
Q

Anything above what line is essentially normal on an audiogram?

A

anything above the 20dB line

41
Q

Audiogram results in sensorineural hearing loss?

A

Both air and bone conduction readings will be more than 20 dB, plotted BELOW the 20 dB line on the chart.

42
Q

Audiogram results in conductive hearing loss?

A

Bone conduction readings will be normal (between 0 and 20 dB).

Air conduction readings will be greater than 20 dB, plotted BELOW the 20 dB line on the chart.

43
Q

What is the most common congenital deformity affecting the orofacial structures?

A

Cleft lip & palate

44
Q

What is there an opening between in a cleft palate?

A

The mouth & nasal cavity

45
Q

What is a risk factor for cleft lip/palate?

A

Maternal epileptic use

46
Q

What infection are those with cleft palate at increased risk of?

A

Otitis media –> referral to 2ary care required

47
Q

What is the 1st priority in Mx of cleft lip/palate?

A

The first priority is to ensure the baby can eat and drink.

48
Q

When is cleft lip surgery vs cleft palate surgery performed?

A

Cleft lip - around 3 months

Cleft palate - around 6-12 months

49
Q

Use of which antiepileptic during prengnacy carries the highest risk of cleft lip or palate?

A

Topiramate

50
Q

What triad of features characterises EBV infection (glandular fever/IM)?

A

1) fever
2) pharyngitis
3) lymphadenopathy

51
Q

What condition can cause palatal petechiae?

A

EBV

52
Q

Lymphadenopathy in IM vs tonsillitis?

A

IM - anterior & posterior triangles of neck

Tonsillitis - typically only results in the upper anterior cervical chain being enlarged

53
Q

In EBV, what antibodies does the body produce?

A

Heterphile antibodies (these can take up to 6 week to be produced).

54
Q

What 2 tests can be used to test for heterophile antibodies in EBV?

A

1) Monospot test
2) Paul-Bunnell test

55
Q

What is the monospot test?

A

This introduces the patient’s blood to RBCs from HORSES.

Heterophile antibodies (if present) will react to the horse RBCs and give a positive result.

56
Q

What are patients advised to avoid in EBV?

A

1) contact sport
2) alcohol

57
Q

What are some complications of IM?

A

1) haemolytic anaemia
2) splenic rupture
3) thrombocytopenia
4) hepatitis
5) cholestastic hepatitis
6) glomerulonephritis

58
Q

EBV infection is associated with certain cancers, notably which one?

A

Burkitt’s lymphoma

59
Q

What is mumps?

A

Mumps is an acute viral illness caused by the Paramyxovirus, primarily affecting the salivary glands, particularly the parotid glands.

60
Q

Give some complications of mumps

A

1) pancreatitis

2) orchitis & oophoritis

3) meningitis & encephalitis

4) sensorineural hearing loss - usually unilateral and transient

61
Q

What is the incubation period of mumps?

A

14-21 days

62
Q

Is mumps a notifiable disease?

A

Yes

63
Q

What virus causes mumps?

A

paramyxovirus

64
Q

What viral load indicates a normal vaginal delivery in HIV?

A

<50 copies/ml

65
Q

What should be given during the c-section if the viral load of HIV is unknown?

A

IV zidovudine

66
Q

What viral load indicates a c-section in HIV?

A

> 50 –> c-section considered

> 400 –> c-section indicated

67
Q

What HIV viral load indicates the need for IV zidovudine during c-section?

A

1) unknown viral load

2) >10000 copies/ml

68
Q

Prophylaxis treatment may be given to the baby depending on the mothers HIV viral load.

What is a ‘low risk baby’? What is a ‘high risk baby’?

A

Low risk –> mothers viral load is <50 copies/ml

High risk –> mothers viral load is >50 copies/ml

69
Q

What HIV prophylaxis is given to low risk babies (i.e. mothers viral load is <50 copies/ml)?

A

Zidovudine for 4 weeks

70
Q

What HIV prophylaxis is given to high risk babies (i.e. mothers viral load is >50 copies/ml)?

A

Zidovudine, lamivudine and nevirapine for 4 weeks

71
Q

Can HIV be transmitted in breastfeeding?

A

Yes, even if the mother’s viral load is undetectable.

72
Q

What can cause a positive HIV result in children <18 months?

A

Positive results may be due to maternal antibodies in children aged under 18 months.

This does not necessarily mean they are HIV positive.

73
Q

How many times are babies to HIV positive parents tested?

A

Twice

74
Q

When are babies to HIV positive parents tested?

A

1) HIV viral load test at 3 months –> if this is negative, the child has not contracted HIV during birth and will not develop HIV unless they have further exposure.

2) HIV antibody test at 24 months –> to assess whether they have contracted HIV since their 3 month viral load e.g. through breast feeding.

75
Q
A

Yr 5 - Paeds (21 decks)

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