Cardio-Resp and Haematology Flashcards

1
Q

What are the symptoms of Von Willebrand disease?

A
Bruising easily
Frequent or long nosebleeds
Bleeding gums
Prolonged bleeding from cuts
Menorrhagia
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2
Q

Which medications help with Von Willebrand disease?

A

Desmopressin
Tranexamic acid
VWF concentrate

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3
Q

What does ITP stand for?

A

Immune thrombocytopenia purpura

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4
Q

What is the pathology behind ITP?

A

Antibodies are made against platelets therefore platelets are removed quickly by the spleen

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5
Q

What are the features of ITP?

A

Bruising or red/purple rashes
Nosebleeds
Menorrhagia
Often occurs 2-3 weeks after an infection

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6
Q

What is the management of ITP?

A

Prednisolone
IV Ig
Platelet transfusion
Splenectomy

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7
Q

Give a differential diagnosis for cyanosis in children

A
Meconium aspiration
Pulmonary atresia/hypertension/stenosis
Tetralogy of fallot
Tricuspid atresia 
Trachea-oesophageal fistula 
Transposition of great arteries
Hypoplastic left heart
Patent ductus arteriosus 
Birth asphyxia 
Transient tachypnoea of newborn
Respiratory distress syndrome
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8
Q

What defects are part of tetralogy of fallot?

A

Pulmonary stenosis
Overriding aorta
VSD
Right ventricular hypertrophy

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9
Q

Give a differential diagnosis for a cough in a child

A
Croup
Asthma
Viral induced wheeze
Pneumonia
Bronchiolitis
Whooping cough/TB
Reflux
CF
Tracheo-oesophageal fistula
Passive smoking
Croup 
Inhaled foreign body 
Post-nasal drip
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10
Q

Give a differential diagnosis for strider in a child

A
Croup 
Quinsy 
Anaphylaxis 
Inhaled foreign body 
Laryngeal anomalies/laryngomalacia 
Upper airway obstruction 
Vascular ring
Tracheal abnormality
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11
Q

Why is epiglottis so rare today?

A

Introduction of HiB vaccine

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12
Q

What is bronchiolitis?

A

An acute obstruction of the small airways usually caused by RSV
Causes respiratory distress and wheeze in infants

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13
Q

When does bronchiolitis tend to occur?

A

Winter

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14
Q

Which virus is most likely to cause bronchiolitis?

A

Respiratory syncytial virus

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15
Q

What might you find on examination of a child with bronchiolitis?

A

Widespread wheeze
Fine crackles
Over expansion of chest

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16
Q

What investigations should you order for suspected bronchiolitis?

A

Bloods
Chest X-ray
Nasopharyngeal aspirate

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17
Q

What is the usual length of disease for bronchiolitis?

A

7-10 days

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18
Q

What is the management of bronchiolitis?

A

Supportive
Oxygen
Bronchodilators- although no evidence for this

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19
Q

What can be given to protect high risk individuals from bronchiolitis?

A

Palivizumab

Monoclonal antibody against RSV

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20
Q

Which organism causes whooping cough?

A

Bordetella pertussis

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21
Q

What is the presentation of whooping cough?

A

Paradoxical coughing spasms during expiration followed by a sharp intake of breath (whoop)

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22
Q

How should we investigate whooping cough?

A

Culture from a nasal swab

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23
Q

What is the management of whooping cough?

A

Supportive

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24
Q

How long can the cough last in whooping cough?

A

Months - 100 days

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25
Q

What is the medical name for croup?

A

Acute laryngotracheobronchitis

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26
Q

Between which ages do children usually get croup?

A

6 months - 3 years

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27
Q

What organism causes croup?

A

Parainfluenza virus

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28
Q

What is the presentation of whooping cough?

A

Coryzal symptoms that proceeds to strider
Barking cough- classically at night
Hoarse voice

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29
Q

What is the management of whooping cough?

A

Self limiting
Observation/reassurance
Steroids if needed

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30
Q

Acute epiglottitis is cause by which organism?

A

Haemophilus influenzae B

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31
Q

What is the presentation of acute epiglottitis?

A

Inability to swallow and talk
Stridor
Barking cough

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32
Q

What is the management of epiglottitis?

A

Intubation

Do not look down throat as this can cause respiratory distress

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33
Q

What is the commonest chronic illness of childhood?

A

Asthma

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34
Q

Name some triggers of asthma

A
Dust mite 
Air pollution
Cigarette smoke
Cold air
Viral infection
Stress
Exercise
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35
Q

How is the clinical diagnosis of asthma made?

A

Recurrent cough/wheeze

Reversible with bronchodilators

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36
Q

What might be seen on a CXR of an asthmatic?

A

Hyperinflation

Areas of collapse - mucus plugging

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37
Q

Describe the stepwise approach to managing asthmatic children

A
SABA
\+ inhaled steroid 
\+ leukotriene antagonist or oral theophylline
increase dose inhaled steroid
\+ oral steroids
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38
Q

What combination of devices should a <5 year old be given for inhaled medications?

A

Metered dose inhaler

Spacer device

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39
Q

What features define a severe asthma attack?

A
Too breathless to talk/feed
RR >30 (>5y/o), RR >40 (2-5 y/o)
HR >120 (>5), HR > 140 (2-5)
PEFR <50%
O2 says <92%
40
Q

Describe the features of life threatening asthma

A
PEFR <33%
O2 sats <92%
Silent chest/cyanosis
Fatigue/drowsy
Confusion
Hypotension
41
Q

How is CF inherited?

A

Autosomal recessive

42
Q

How many people are carriers for cystic fibrosis?

A

One in 25

43
Q

What is the pathophysiology of cystic fibrosis?

A

A molecular defect in the cellular membrane chloride channel leads to production of excessively thick mucus in many systems

44
Q

What is the defining test for cystic fibrosis?

A

Sweat test >60 mmol/L

45
Q

Which chromosome is affected in cystic fibrosis?

A

Chromosome seven

46
Q

What percentage of cystic fibrosis is due to the Delta F508 gene mutation?

A

75%

47
Q

How is cystic fibrosis usually diagnosed?

A

The heel prick test

Or antenatally in an affected family

48
Q

How might an infant present with cystic fibrosis?

A

Meconium ileus

Failure to thrive and malabsorption

49
Q

Which two organisms are more likely to occur in cystic fibrosis patients?

A

Pseudomonas

Burkholderia cepacia

50
Q

How do you manage the respiratory symptoms in cystic fibrosis?

A
Regular bronchodilators
Antibiotics and steroids
Nebulised DNase
Preventative physiotherapy
Prophylactic immunisations
51
Q

What are the consequences of pancreatic failure in cystic fibrosis?

A

Malnutrition and deficiency in fat soluble vitamins

A DE and K

52
Q

How do you manage the GI effects of CF?

A

Creon
Enzyme capsules taken with food to aid absorption
High calorie diet
Fat soluble vitamin supplements

53
Q

What percentage of CF patients will develop an impaired glucose tolerance?

A

25%

54
Q

What is the effect of cystic fibrosis on the liver?

A

A decreased rate of bile flow leading to biliary disease and cirrhosis

55
Q

What medication helps with the liver effects of CF?

A

Ursodeoxycholic acid

56
Q

What is the most common childhood cancer?

A

Leukaemia

Acute lymphoblastic leukaemia

57
Q

What is the most common type of leukaemia in children?

A

Acute lymphoblastic leukaemia

58
Q

How does leukaemia present in children?

A

Malaise, anaemia, bruising, bone pain and lymphadenopathy

59
Q

What kind of therapy is used for leukaemia?

A

Chemotherapy

60
Q

What is the overall prognosis for the acute leukaemias in childhood?

A

ALL is 85% survival

AML is 60 to 70% survival

61
Q

Define leukaemia

A

A malignant proliferation of white cell precursor is in marrow
Blast cells escape into circulation and maybe deposited in lymphoid all of the tissue

62
Q

What is the peak age group for acute lymphoblastic leukaemia?

A

2 to 5 years old

63
Q

What will the blood show in an acute leukaemia?

A

Anaemia
Thrombocytopenia
Increased white cells
Blast cells seen on peripheral blood film

64
Q

How is the diagnosis of leukaemia confirmed?

A

Bone marrow aspirate

65
Q

Tumour lysis syndrome causes an increase of which metabolites?

A

Urate
Phosphate
Potassium

66
Q

How do we prevent the tumour lysis syndrome?

A

Good hydration

Allopurinol

67
Q

Give some late consequences of treatment for leukaemias

A

Short stature
Delayed puberty/growth hormone deficiency/hypothyroidism/gonadal failure
Neuro cognitive effects
Subfertility, nephrotoxicity, deafness, pulmonary fibrosis, cardiomyopathy-chemotherapy

68
Q

Give some causes of normocytic normochromic anaemia

A

Chronic renal disease
Hypothyroidism
Chronic inflammatory disease
Chronic infection

69
Q

Why is iron deficiency anaemia common in childhood?

A

High demand for iron due to rapid growth

Poor intake of iron rich food

70
Q

How is breastmilk protective for iron deficiency anaemia?

A

Iron binding protein lactoferrin is found in breastmilk therefore iron is absorbed more efficiently

71
Q

Describe the blood film showing iron deficiency anaemia

A

Microcytosis
Hypochromia
Poikilocytosis - variation in cell shape

72
Q

What type of thalassaemia is most common?

A

Beta

73
Q

Which type of thalassaemia may be confused with iron deficiency anaemia and why?

A

Heterozygous - beta trait

Mild hypochromic, microcytic anaemia

74
Q

How is thalassaemia diagnosed?

A

Haemoglobin electrophoresis

Increased HbA2 and HbF

75
Q

What is the management of beta thalassaemia?

A

Regular blood transfusions

76
Q

What are the complications of having thalassaemia?

A

Haemosiderosis due to iron overload
Cardiomyopathy
Diabetes
Skin pigmentation

77
Q

Describe the pathophysiology of sickle-cell anaemia

A

One amino acid substitution in the gene
This causes unstable haemoglobin which when in the deoxygenated state is a highly structured polymer that causes brittle red blood cells
This can occlude vessels and cause ischaemia

78
Q

What can precipitate crises of sickle-cell anaemia?

A

Dehydration
Hypoxia
Infection
Acidosis

79
Q

What is the management of the sickle-cell crisis?

A

Painkillers
Fluids
Warmth
Antibiotics if required

80
Q

If an infant is still cyanosed after stabilisation what should you give?

A

Prostaglandin E

To keep the ductus arteriosus open

81
Q

Which congenital heart conditions give a cyanotic appearance?

A

Transposition of the great vessels
Hypoplastic left or right heart
Tetralogy of Fallot
Critical pulmonary stenosis

82
Q

How is the patient kept alive with transposition of the great vessels before surgery?

A

Patent ductus arteriosus or ventricular septal defect

83
Q

When would you operate on a baby with transposition of the great arteries?

A

Day one or two

84
Q

What is the most common congenital cyanotic heart disease?

A

Transposition of the great vessels

85
Q

How does tetralogy of fallot become a cyanotic heart disease?

A

If the pulmonary stenosis is critical

86
Q

What murmurs can be heard in a baby with the tetralogy of fallot?

A

Pulmonary stenosis-ejection systolic that radiates to the left shoulder or back
Ventricular septal defect-pansystolic murmur that is heard at the left sternal edge

87
Q

What needs to be done for a baby with tetralogy of fallot?

A

Balloon dilatation of the pulmonary stenosis and correction of the ventricular septal defect

88
Q

What are Tet spells?

A

Occur if a patient with TOF is dry or dehydrated
The pulmonary vessels are narrowed and vascular shuts down
need an increased pressure to reopen
Causes symptoms of pain, crying and poor feeding

89
Q

How do we treat a Tet spell?

A

Put knees up to the chest to increase the pulmonary flow
Morphine
Oxygen
Fluids

90
Q

What is the most common congenital heart defect?

A

Ventricular septal defect

91
Q

How does heart failure present in a child?

A
Poor feeding
Shortness of breath on feeding
Tiredness
Increased sweating
Oedema-pitting legs and crackles on chest
92
Q

Describe the murmur of a patent ductus arteriosus

A

Systolic, constant, machinery like

93
Q

Describe the murmur of an atrial septal defect

A

Ejection systolic murmur

Splits the second and first heart sounds

94
Q

What are the viral causes of pneumonia in children?

A
RSV
Influenza
Parainfluenza
Adenovirus
Coxsackie virus
95
Q

What is the most common type of bacterial pneumonia in a newborn?

A

Group B beta haemolytic strep

96
Q

What do you find on examination of a pneumonia?

A

Dullness to percussion
Bronchial breathing
Crackles

97
Q

What is the first antibiotic for community-acquired pneumonia?

A

Amoxicillin

Or erythromycin