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Year 4 Paediatrics > Paediatric Haematology > Flashcards

Paediatric Haematology Flashcards

1
Q

Feotal haemoglobin structure

A

2 alpha and 2 gamma units - greater affinity to oxygen

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2
Q

Hydroxycarbamide

A

Used to increase the production of HbF in sickle cell patients

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3
Q

Why do sickle cell patients present after 1 years old

A

Sickle cell involves a defect in the beta haemoglobin - before 1 years old, infants still have some HbF which has a greater affinity for oxygen

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4
Q

Causes of anaemia in infancy

A
  • Physiological
  • anaemia of prematurity
  • blood loss
  • haemolysis
  • twin to twin transfusion
  • haemolysis - spherocytosis, G6PD deficiency, haemolytic disease of the newborn
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5
Q

Physiological anaemia

A

Normal dip in haemoglobin between 6 - 9 weeks due to high oxygen delivery to the tissues causing negative feedback

Less erythropoietinproduction by the kidneys

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6
Q

Causes of anaemia of prematurity

A

Become anaemic due to:

  • neonatal sepsis
  • less time in utero recieving iron from mother
  • RBC production cannot keep up with rapid growth in first few weeks
  • Reduced erythropoietin levels
  • Frequent blood tests
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7
Q

Pathophysiology of haemolytic anaemia of the newborn

A

Caused by incompatibility between the rhesus antibodies of the mother and rhesus antigens of the baby

Occurs in a rhesus negative mother and rhesus positive second child

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8
Q

Test for haemolytic disease of the newborn

A

Direct Coombs test

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9
Q

Management of haemolytic disease of the newborn

A

Baby is given phototherapy if jaundiced

May need blood transfusion

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10
Q

Prevention of haemolytic disease of the newborn

A

Mothers are given RhoGAM during pregnancy which is a rhesus immunoglobulin

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11
Q

Causes of anaemia in older children

A

Iron deficiency - dietary insufficiency
Blood loss - menstruation in girls

Sickle cell anaemia
Thalassaemia 
Leukaemia
Hereditary spherocytosis or eliptocytosis 
Sideroblastic anaemia
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12
Q

Presentation of anaemia

A 
Fatigue and lethargy 
SOB on exertion
Headaches 
Dizziness 
Palpitations
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13
Q

Causes of microcytic anaemia

A

TAILS:

Thalassaemia 
Anaemia of chronic disease 
Iron deficiency 
Lead poisoning 
Sideroblastic anaemia
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14
Q

Causes of normocytic anaemia

A

3 As + 2 Hs

Anaemia of chronic disease
Acute blood loss
Aplastic anaemia

Haemolytic anaemia
Hypothyroidism

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15
Q

Causes of macrocytic anaemia

A

FAT - L

Folate and B12 deficiency
Alcohol
Thyroid disease - hypothyroidism
Liver disease

Others:

  • Azathioprine
  • Reticulocytosis
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16
Q

Symptoms specific to iron deficiency

A

Pica - weird dietary cravings

Hair loss

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17
Q

Signs of anaemia

A

Pallor
Tachycardia
Pale conjunctiva
Raised RR

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18
Q

Signs of iron deficiency

A

Koilonychia - spooned nails
Angular chelitis
Atrophic glossitis

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19
Q

Investigations for anaemia

A 
FBC 
Blood film if required 
Reticulocyte count 
Iron - ferritin 
B12 and folate 
Bilirubin- if jaundice or haemolysis suspected

Direct Coombs test - autoimmune haemolytic anaemia
Haemoglobin electrophoresis- haemoglobinopathies

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20
Q

Causes of iron deficiency

A

Inadequate dietary intake
Menstruation
Malabsorption - Crohn’s and Coeliacs, PPI use

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21
Q

Blood tests for iron

A 
Serum ferritin (Fe2+) 
Serum Iron - not useful on its own as varies throughout the day 
Total iron binding capacity - space on transferrin molecules for iron to bind 
Transferrin saturation ( serum iron/ TIBC)
22
Q

What increases free iron

A

Supplementation with iron
Acute liver damage - as iron is stored in the liver
Inflammation

23
Q

Side effects of iron supplements

A

Constipation

Black stools

24
Q

Leukaemia

A

Unregulated production of either myeloid or lymphoid blood cells due to cancer of the bone marrow

25
Q

Types of leukaemia

A

Acute lymphoblastic leukaemia - most common in children
Acute myeloid leukaemia
Chronic myeloid leukaemia - rare

26
Q

In which age groups are acute lymphoblastic leukaemia and acute myeloid leukaemia seen in?

A

Acute lymphoblastic leukaemia - 2 - 3 years old

Acute myeloid leukaemia - under 2 years old

27
Q

Risk factors for leukaemia

A 
Radiation exposure during pregnancy 
Down’s syndrome 
Kleinfelter syndrome 
Noonan syndrome 
Fanconi’s anaemia
28
Q

Presentation of leukaemia

A 
FLAWS - fever, lethargy, appetite loss, weight loss, sweating at night 
Failure to thrive 
Pallor - anaemia 
Petechiae + abnormal bruising and bleeding - thrombocytopenia 
Abdominal pain 
Generalised lymphadenopathy 
Joint or bone pain 
Hepatosplenomegaly
29
Q

Diagnosing leukaemia

A

Refer any child with unexplained petechiae or hepatomegaly for immediate specialist assessment

Urgent (within 48 hours) FBC - anaemia, thrombocytopenia, leukopenia
Blood film - blast cells
Bone marrow biopsy
Lymph node biopsy

Staging CT scan, chest Xray, LP, genetic analysis using imunnophenotyping

30
Q

Management of leukaemia

A

MDT
- paediatric oncologist - chemotherapy

Other therapies:

  • radiotherapy
  • bone marrow transplant
  • surgery
31
Q

Complications of chemotherapy

A 
Failure to treat leukaemia 
Stunted growth and development 
Immunodeficiency- recurrent infections
Neurotoxicity 
Infertility 
Secondary malignancy 
Cardiotoxicity
32
Q

Prognosis for acute lymphoblastic leukaemia

A

80% cure rate

33
Q

Pathophysiology of idiopathic thrombocytopenic purpura

A

Type II sensitivity reaction causing autoimmune destruction of plateletes

34
Q

Presentation of idiopathic thrombocytopenic purpura

A

Presents in children under 10 years old often after a viral illness

Symptoms present 1 - 2 days after virus:

  • bleeding - epistaxis, gums or menorrhagia
  • bruising
  • petechial or purpuric rash
35
Q

Management of idiopathic thrombocytopenic purpura

A

If actively bleeding or severe thrombocytopenia (<10mmol/l)

  • prednisolone
  • IV immunoglobulins
  • blood transfusion if required
  • platelet transfusion if required

Education:

  • avoid contact sport
  • avoid IM injections and lumbar punctures
  • avoid NSAIDs, aspirin and blood thinning medication
  • advice on managing nosebleeds
  • seek help after an injury that may cause internal bleeding
36
Q

Investigations for idiopathic thrombocytopenic purpura

A

Bloods - FBC - thrombocytopenia

Blood culture to rule out sepsis

37
Q

Complications of idiopathic thrombocytopenic purpura

A

Chronic idiopathic thrombocytopenic purpura (ITP)
Anaemia
Intracranial and subarachnoid haemorrhage
GI bleeding

38
Q

Sickle cell anaemia pathophysiology

A

Abnormal gene for beta haemoglobin on chromosome 11 (glut - valine substitution) causing sickling and haemoglobin S which is prone to haemolysis

39
Q

Sickle cell trait

A

Carrier but not symptomatic

40
Q

Sickle cell inheritance pattern

A

Autosomal recessive

41
Q

How to diagnose sickle cell anaemia

A

Heel prick test at 5 days old

Testing during pregnancy for high risk mothers

42
Q

Complications of sickle cell anaemia

A

Increased risk of infection
Stroke
Avascular necrosis especially in the hip
Pulmonary hypertension
Painful and persistent penile erection (priapism)
CKD
Sickle cell crisis

43
Q

General management for sickle cell

A

Stay hydrated and avoid triggers for crises
Ensure vaccinations are up to date
Antibiotic prophylaxis
Hydroxycarbamide - stimulates production of HbF
Blood tranfusion - severe anaemia
Bone marrow transplant

44
Q

Types of crisis

A

Vaso - occlusive crisis (painful crisis)
Splenic sequestration crisis
Aplastic crisis

45
Q

Vaso - occlusive crisis

A

HbS (sickled Hb) blocks capillaries and causes distal ischaemia

  • associated with dehydration and raised haematocrit

Symptoms:

  • pain
  • fever
  • priapism
46
Q

Management of priapism

A

Urological emergency

Treated with apiration of the blood from the penis

47
Q

Splenic sequestration crisis

A

Caused by HbS blocking blood flow within the spleen causing acute and painful splenomegaly
Pooling of blood within the spleen can cause severe anaemia and hypovolaemic shock

Emergency - blood tranfusion and fluod resuscitation to treat anaemia and shock

Recurrent crises - splenectony

48
Q

Aplastic crisis

A

Temporary loss of the creation of new red blood cells - often due to parvovirus B19

Causes significant anaemia - often requiring blood transfusions. Can resolve spontaneously within a week

49
Q

Acute chest syndrome

A

Fever/resp symptoms + new inflitrates seen on chest Xray

Can be due to infection e.g. pneumonia or bronchiolitis or pulmonary vaso - occlusion

50
Q

Treatment of acute chest syndrome

A

Emergency

Antibiotics or antivirals
Blood transfusion - anaemia
Incentive spirometry - encourages deep breathing
Artificial ventilation - NIV/ intubation

Year 4 Paediatrics (19 decks)

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