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paediatrics > haematology > Flashcards

haematology Flashcards

1
Q

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease signs and symproms

A

aneamia
hepatosplenomegaly
jaundice
CCF

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2
Q

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease management

A

anti D immunoglobulin to Rh -ve mothers

intrauterine transfusion of affected fetuses

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3
Q

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease DD

A

thalassaemia

infection - toxoplasmosis, CMV

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4
Q

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease diagnosis

A

coombs test - Rh -ve mothers tested for D antibodies

kleihauer test

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5
Q

Anaemia of Prematurity cause

A

Low birth weight infants

poor erythropoietin response

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6
Q

Anaemia of Prematurity signs and symptoms

A 
apnoea
poor weight gain
pallor
decreased activity
tachycardia
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7
Q

iron deficiency anaemia causes

A

Poor intake + increased requirement.
blood loss
malabsorption

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8
Q

iron deficiency anaemia presentation

A 
pallor
irritability
anorexia when Hb<50,
tachycardia
splenomegaly
if recurrent, suspect bleeding
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9
Q

iron deficiency anaemia management

A

oral iron - ferrous fumarate for 3-6 months

constipation common

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10
Q

Sickle Cell Disease management

A 
Prophylactic penicillin 
Pneumococcal, influenza, meningococcal
vaccines
high risk of sepsis
pain management - paracetamol, NSAIDS, O2
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11
Q

Sickle Cell Disease complications

A 
acute anaemia (aplastic crisis)
vaso-occlusive crisis - cold, exercise, infection
cardiomegaly (high output)
strokes
sepsis
asplenia - from filtering abnormal RBC
splenomegaly
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12
Q

Sickle Cell Disease aplastic crisis cause

A

parovirus B19

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13
Q

Sickle Cell Disease stroke management

A

long term transfusion therapy

chelation for iron overload if transfusion long term

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14
Q

β Thalassaemia Minor signs

A

asymptomatic
mild anaemia
low MCV
Raised Hb A2

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15
Q

β Thalassaemia Major signs and symptoms

A 
Progressive Severe Anaemia
low MCV, 
Hb F and A2 increased
Jaundice
Splenomegaly
Failure to thrive
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16
Q

β Thalassaemia management

A

Genetic Counselling

Regular blood transfusion

17
Q

G6PD presentation

A

Neonatal jaundice
Chronic non-spherocytic haemolytic anaemia
Intermittent episodes of intravascular haemolysis
also dark urine (urobillinogen and haemoglobulinuria)

18
Q

G6PD what is it

A

RBC lack G6PD so exposed to oxidant induced haemolysis

19
Q

hereditary spherocytosis

A

RBC lose part of membrane, reducing surface:volume. cells become spheroidal leads to destruction

20
Q

hereditary spherocytosis presentation

A

autosomal dominant
jaundice
anaemia
splenomegaly

21
Q

Diamond Blackfan anaemia presentation

A 
physical anomalies- at least 50%
• Cranio-facial
• thumb 10-20%
• Deafness
• Musculoskeletal
22
Q

Immune / Idiopathic Thrombocytopaenic Purpura (ITP)

A

platelet destruction by Ig G autoantibodes
usually post viral
acute brusing, looks dramatic rarely dangerous

23
Q

von willebrand disease causes

A

deficiency in vwf, a carrier protein for factor 8

24
Q

von willebrand disease management

A

Tranexamic acid

25
Q

haemophilia A

A

Deficiency of Factor VIII

26
Q

haemophilia B

A

Deficiency of Factor IX, ↑APTT

27
Q

haemophilia A/B presentation

A 
X linked recessive- boys
Prolonged bleeding
Muscle bleeds
Joint bleeds > arthritis and deformity
Treatment Factor VIII/IX
28
Q

acute lymphoblastic leukaemia presentation

A 
peak age 4-7
normochromic normocytic anaemia (pallor)
thrombocytopenia (bleeding)
leukopenia (infections)
fatigue
weight loss
painless lumps on neck (lymphadenopathy)
29
Q

acute lymphoblastic leukaemia diagnosis

A

bone marrow biopsy
tdt and cd10 surface markers in immunophenotyping
CT/MRI and CXR

30
Q

acute lymphoblastic leukaemia complications

A

neutropenic sepsis - tazocin and gentamicin
poor growth
second malignancies

31
Q

acute lymphoblastic leukaemia management

A 
Induction - vincristine, dexamethasone
Consolidation and CNS treatment (if known CNS disease)
Intensification
Maintenance
(radio / chemotherapy)
32
Q

fanconi anaemia

A

autosomal recessive
most common cause of inherited aplastic anaemia
high risk of leukaemia
bone marrow transplant needed

33
Q

anaemia range

A

<110g/L

34
Q

microcytic hypochromic
normocytic normochromic
macrocytic

ranges and causes

A

MCV <70 iron def, thalassaemia

MCV 81-97 haemolysis (sickle cell)

MCV >100 B12 def (pernicious/malabsorption), folate def (malnutrition)

35
Q

effects of ALL treatment

A 
fertility
growth
psychological
puberty
intellectual
36
Q

ALL good prognostic factors

A

Age 2-10
Female
WCC<50
No CNS disease

prognosis 80%

paediatrics (13 decks)

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