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Finals - Paediatrics > Haematology (paeds) > Flashcards

Haematology (paeds) Flashcards

1
Q

What are some causes of severe anaemia at birth?

A

Haemolytic disease of the newborn/erythroblastosis fetalis

Bleeding - umbilical cord, internal haemorrhage

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2
Q

What is the pathophysiology of erythroblastosis fetalis and how does it manifest clinically?

A

Rh negative mother previously sensitised to Rh positive cells (e.g. from first pregnancy)

Transplacental passage of anti Rh antibodies - haemolysis of Rh+ve foetal cells

Severe anaemia with compensatory hyperplasia/enlargement of liver and spleen

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3
Q

How do you manage erythroblastosis fetalis?

A

Intrauterine blood transfusions of affected foetuses

Every 1 to 2 weeks, usually until 32 to 35 weeks

Delivery planning

Possible postnatal transfusions if not given intrauterine

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4
Q

How do you prevent erythroblastosis fetalis?

A

Can give mother
Rh immunoglobulin - reduce a mother’s reaction to their baby’s Rh-positive blood cells

Administered at around the 28th week of pregnancy and again at least 72 hours after birth if the baby is Rh positive

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5
Q

What is physiological anaemia of the newborn?

A

Natural fall in Hb from birth - nadir reached at 2m

Due to decreased RBC production, plasma dilution secondary to increasing blood volume, shorter life span of foetal RBCs (50-70days), more fragile RBCs, switch from HbF to HbA

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6
Q

What is anaemia of prematurity?

A

Low birth weight infants have a poor erythropoetin (renal production) response - dont stimulate bone marrow to grow enough RBCs

Protein content of breast milk may also be too low for effective haemopoesis in the premature infant

Hb levels rapdily decline after birth, lowest at 6wks of age

Presents with: 
Apnoea
Poor weight gain
Pallor 
Decreased activity 
tachycardia
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7
Q

How does the source of iron supply differ between childhood and adulthood?

A

Children:
Diet = 30%
Recycled from red cells = 70%

Adults:
Diet = 5%
Recycled from red cells = 95%

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8
Q

What are some risk factors for iron deficiency anaemia in childhood?

A 
Not delaying umbilical cord clamping/cutting by 1min
Low birth weight 
Excess cows milk intake 
Cow's milk intolerance 
Occult GI bleed e.g. hookworm
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9
Q

How does iron deficiency anaemia in childhood present?

A 
Pallor
Irritability
Anorexia when Hb<50
Tachycardia, cardiac dilatation, murmur 
Possible splenomegaly

Really low iron levels (and consequently low Hb) may present with pica = compulsions to consume inorganic matter e.g. ice, soil, hair, paper, metal, glass, sharp objects

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10
Q

What are the blood results for iron deficiency anaemia?

A

Microcytic, hypochromic anaemia

Low-normal reticulocytes; pencil/cigar cells on blood film

Low ferritin and serum iron; Increased TIBC

High ZPP - If there is not enough iron available, then protoporphyrin combines with zinc instead of iron to form zinc protoporphyrin

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11
Q

How do you manage iron deficiency anaemia?

A

Supplementary iron

6mg/kg/day PO

Hb levels increase at c.10g/L/wk

Iron stores replenished by 3months - maintain treatment for 3-6m total

SE: constipation most common

Treatment failure: non-compliance

Also address diet where appropriate

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23
Q

What is the aetiology and pathophysiology behind thalassaemias?

A

Reduced globin chain synthesis:

Normal = 2xalpha + 2xbeta

Beta thal = HbF (alpha + gamma) and HbA2 (alpha
+ theta

Alpha thal = 4x alleles - 1-2 lost = asymptomatic; 3-4 lost or Hb beta4 (alpha thal major)

NONE OF THIS IS CLEAR ON THE SLIDES SO AND I CBA

24
Q

How does beta thalassaemia minor present?

A

Asymptomatic

Mild microcytic anaemia

Raised Hb A2

25
Q

How does beta thalassaemia major present?

A

Progressive Severe
Microcytic anaemia

Hb F and A2 increased

Jaundice
Splenomegaly
Failure to thrive
Skeletal Deformity
Delayed puberty
Death early teens/adulthood
26
Q

How do you mange beta thalassaemia major ?

A

Genetic Counselling, AN diagnosis

Regular blood transfusion
Manage iron overload - chelation
Bone Marrow Transplantation

27
Q

What is G6PD deficiency?

A

Genetic disorder - lack of glucose-6-phosphate dehydrogenase which is essential for RBC functioning

Presentation:
Neonatal jaundice
Chronic non-spherocytic haemolytic anaemia
Intermittent episodes of intravascular haemolysis - precipitated by: drugs, fava beans, fever, acidosis

Presentation/investigation:
Rigors, fever, back pain
Haemoglobinuria
Hemighosts, blister cells (precursors to..) and bite cells on blood film

Management:
Stop precipitant
Transfusion
Renal support

28
Q

Stuff on hereditary spherocytosis, bone marrow failure (Parvovirus, Transient erythroblastopenia of
childhood, Diamond-Blackfan anaemia -usually <1yr)

A

CBA

29
Q

What is idiopathic/immune thrombocytopaenic purpura? (ITP)

A

Most common form of immunologic
thrombocytopenia

Acute + chronic, acute often following viral illness, usually resolving in 1-3 mo

Presentation:
Petechiae on dependent extremities is main
expression in childhood acute ITP
Bleeding e.g. GI, nose, gingivae, haematuria, bloody stools, menorrhagia
No splenomegaly or neutropaenia
Rarely dangerous

Management:
Often self-recovering - wait and see
May need tranexamic acid (antifibrinolytic - reduces conversion of plasminogen to plasmin - retention of clots, less bleeding)
Sometimes IVIg, steroids or splenectomy

30
Q

What is von Willebrand disease?

A

Bleeding disorders caused by an abnormality of
the von Willebrand factor (vWF), carrier protein
for Factor VIII – can range from almost undetectable to severe bleeding propensity

31
Q

What is the function of vWF?

A

Binds on platelets to its specific receptor glycoprotein Ib and acts as an adhesive bridge between the platelets and damaged subendothelium at the site of vascular injury – i.e. causes platelets to stick

Also protects FVIII from degradation

32
Q

What are the types of von Willebrand disease?

A

Type 1:
70-80% of vWFD Quantitative deficiency in vWF
Autosomal dominant, variable penetrance
Generally mild, can be asymptomatic and vary with time

Type 2A + 2B: 
C.15% 
Abnormal vWF
AD
Moderate severity 
Type 3:
Rare
Low vWF and Factor VIIIc in plasma, no vWF on platelets 
AR - more common in consanguinuity 
Most severe
33
Q

How does vWFD present? How is it investigated and treated?

A

History:
Often mild bleeding (e.g. bruising, epistaxis, primary
menorrhagia)

Investigation:
Clotting screen may be normal or activated partial thromboplastin time/APTT increased

vWF and Factor VIII variably decreased

Treatment:
If severe bleeds -surgery
Tranexamic acid
Desmopressin/DDAVP usually increases vWF and Factor VIII
Factor VIII/VWF plasma concentrates for severe

34
Q

What are the features of haemophilia A and B?

A

Deficiency of Factor VIII/IX, ↑APTT

X linked recessive- boys

Presentation:
Easy bruising
Prolonged bleeding, Muscle bleeds, mouth bleeds (e.g. following dentist)
Joint bleeds > arthritis and deformity

Treatment:
Exogenous Factor VIII/IX
Desmopressin

35
Q

What is the epidemiology of paediatric leukemia?

A

Most common types of cancers in children

85% ALL:
Peaks between 4-7yrs
Prognosis = 80% cured
Good prognostic factors = 2-10yrs, female, WCC <50, no CNS disease

13% AML

2% other e.g. CML

36
Q

How do leukemias present?

A 
Bone marrow deficits: 
Anaemia - palpitations, dizziness etc
Infections - recurrent, uncommon bugs 
Bleeding/easy bruising 
Weight loss, fever, night sweats, lymphadenopathy 
Hepatosplenomegaly  
Bone pain

Symptoms may come on over a few weeks or a non-specific prodrome over several months

37
Q

What are the blood results for ALL?

A

Anaemia
WCC high or low or normal but neutropaenia usually present
Thrombocytopaenia
Presence of blast (lymphoblast) cells

Bone marrow biopsy is diagnostic

LP - see if has spread to CSF

38
Q

How do you manage ALL?

A

Stratified into risk groups - ABC

Chemotherapy:

Induction -

Consolidation -

Intensification -

Maintenance -
2yrs in girls
3yrs in boys

39
Q

What are some late effects of treatment?

A

Psychological, familial, social

Development - physical (growth problems) and intellectual difficulties

Puberty - potentially delayed

Fertility - infertile due to irradiation or chemotherapy damage to gonadal tissue

Second malignancy - leukemias, lymphomas (due to radiotherapy radiation)

Finals - Paediatrics (33 decks)

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