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Y5 Paediatrics > Haem > Flashcards

Haem Flashcards

1
Q

What is Hb like in neonates?

A

HIGH

to compensate for low oxygen concentration in utero

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2
Q

Why does Hb in neonates progressively fall?

A

Due to decreased RBC production

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3
Q

What are normal Hb ranges for neonate / infant / child?

A

neonate: >140
infant (1m-1y): >100
Child (1y-12y) >110

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4
Q

What are mechanisms of anaemia split into

A

Reduced production

  • ineffective erythropoiesis (e.g. iron deficiency)
  • red cell aplasia (NO PRODUCTION)

Increased destruction (haemolysis)

Blood loss

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5
Q

What are causes for red cell aplasia

A

Parvovirus B19

Diamond-Blackfan

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6
Q

What are causes for ineffective erythropoiesis

A

Iron deficiency
folic acid deficiency
Chronic inflammation, chronic renal failure

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7
Q

What are causes of haemolysis

A

RBC membrane disorder (e.g. hereditary spherocytosis)
RBC enzyme disorder (e.g. G6PD deficiency)
Haemoglobinopathy (thalassaemia, sickle cell)
Immune (haemolytic disease of newborn, AI haemolytic anaemia)

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8
Q

What are main causes of iron deficiency anaemia in neonates?

A

inadequate intake
malabsorption
blood loss

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9
Q

What are sources of iron for infants

A

breast milk
infant formula
cows milk
solids e.g. cereal

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10
Q

what are clinical fts of iron deficiency anaemia in the newborn

A 
Asymptomatic until Hb<60 
Fatigue 
Slow feeding 
Pale 
Pica (inappropriate eating of non-food e.g. soil)
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11
Q

What are investigations for iron deficiency anaemia

A

Blood film: microcytic hypo chromic anaemia (low MCV, low MCH)

Iron studies (ferritin low, serum iron low, TIBC high)

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12
Q

What is management for iron deficiency anaemia?

A

Dietary advice

Ferrous sulphate PO

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13
Q

How do you monitor iron deficiency anaemia

A

Check Hb after 4 weeks - levels should rise by 2g/100
Otherwise check compliance

Once Hb levels are normal, continue iron tx for 3 months to replenish stores

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14
Q

What are specific tests for hereditary spherocytosis

A

Dye binding assay

Osmotic fragility test

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15
Q

How do you manage hereditary spherocytosis:

A

Supportive
RBC transfusion
Folic acid supplementation

Consider phototherapy / exchange transfusion if baby also has jaundicer

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16
Q

What is G6PD deficiency

A

G6PD > rate limiting step in pentose phosphate pathway > necessary to prevent oxidative damage to RBC

Deficiency means they are susceptible to oxidants > haemolysis

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17
Q

What are triggers for G6PD deficiency haemolysis

A

Antimalarials
Antibiotics
Alnalgesics (aspirin)
Chemicals (Naphtaline, fava beans)

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18
Q

What is pattern of inheritance of G6PD

A

X linked recessive

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19
Q

What are clinical features of G6PD

A

NEONATAL JAUNDICE
Trigger
Pallor
Dark urine with haemoglobin AND urobilinogn

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20
Q

How do you diagnose G6PD

A

measure G6PD activity in blood

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21
Q

What is haemolytic disease of the newborn

A

Haemolysis due to antibodies against blood group antigens (ABO/RhD)

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22
Q

How do you identify haemolytic disease of the newborn

A

COOMBS TEST +

Essentially picks up if there are any RBC circulating with antibodies on them

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23
Q

What are main causes of blood loss in the foetus / newborn

A

maternal occult haemorrhage

TTTS

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24
Q

Why does anaemia of prematurity occur

A

Inadequate EPO production
Low RBC lifespan
Frequent blood sampling
Iron / folate deficiency

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25
Q

What is another word for bone marrow failure

A

APLASTIC ANAEMIA

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26
Q

What are 2 inherited causes of aplastic anaemia

A

Fanconi

Schwachmai-Diamond Syndrome

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27
Q

Explain Fanconi Anaemia

A

TRIAD

  • congenital abnormalities
  • defective haematopoesis
  • high risk AML/Tumours
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28
Q

What are the congenital abnormalities in Fanconi Anaemia

A

NORD

NEURO

  • micropthalmia,
  • microcephaly,
  • developmental delay

ORTHO:
- short stature, hip dislocation, scoliosis, SHORT THUMB

RENAL

  • renal aplasia/hypoplasia
  • horseshoe kidney
  • double ureter

DERM

  • cafe au last spots
  • hypo/hyperpigmented
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29
Q

How do you diagnose Fanconi anaemia

A

increased chromosomal breakage of peripheral blood lymphocytes

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30
Q

How do you manage Fanconi

A

BM transplant

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31
Q

What is the triad in Schwachman-Diamond Syndrome

A

TRIAD Schwachman-Diamond Syndrome:

  • BM failure
  • pancreatic failure
  • skeletal abnormality
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32
Q

Give examples of inherited bleeding disorders

A

haemophilia

vWF deficiency

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33
Q

Give examples of congenital clotting disorders

A

protein C deficiency
Protein S deficiency
Antithrombin deficiency
FV leiden

34
Q

What is mode of inheritance for haemophilia

A

X linked recessice

35
Q

What is hallmark feature of haemophilia

A

Recurrent DEEP bleeding into joints, muscles

36
Q

What is a bad consequence of haemophilia

A

arthritis

37
Q

How do you manage haemophilia

A

Recombinant F8 for HA
Recombinant F9 for HB

Also give prophylactic F8 if severe HA to reduce risk of joint damage

Also Desmopressin (DDAVP) in mild haemophilia A

38
Q

What is hallmark feature of vWD

A

SUPERFICIAL BLEEDING

epistaxis, menorrhagia

39
Q

How do you manage vWD

A

Type 1 (poor quality): DDAVP

T2/3: plasma derived F8 concentrated

40
Q

What must you avoid in vWD/haemophilia

A

IM injections
aspirin
NSAIDS

41
Q

What is a dangerous consequence of vitamin K deficiency in babies

A

Haemorrhagic disease of the newborn

42
Q

What is vitamin K necessary for

A

F2, 7, 9, 10

Protein C, S production

43
Q

What is the cause of ITP

A

Antiplatelet IgG antibodies

Cause destruction of circulating platelets

44
Q

How does ITP present

A

In children, few weeks after viral infection
Children develop petechiae, purpura, superficial bruising
Epistaxis, mucosal bleeding

*** beware of intracranial bleeding

45
Q

How do you manage mild ITP

A

self resolving

manage at home with corticosteroids or IVIG

46
Q

How do you manage severe ITP

A

IVIG, corticosteroids, platelet transfusion

47
Q

Explain how MAHA occurs

A

MECHANISM NOT DISEASE
clot forms in blood vessel > blood cannot flow nicely > sheared into small fragments > schistocytes on blood film + anaemia, jaundice

48
Q

Explain how HUS occurs

A

E coli 0157:H7
produces shiga-like toxin
Toxin damages endothelium of glomerular vessel
causes platelet aggregation > thrombocytopenia
shearing of RBC > MAHA
less end organ perfusion > RENAL failure

49
Q

What is the triad of HUS + other symptom to remember

A

MAHA + THROMBOCYTOPOENIA + RENAL FAILURE

+ diahrroea

50
Q

How does TTP occur

A

antibodies against ADAMTS13
causes vWF to stick together > clots form > platelets all stuck together

cause same as above + diminished end organ perfusion to brain as well, causing perfusion

51
Q

What is PENTAD of TTP

A

MAHA + RENAL FAILURE + THROMBOCYTOPOENIA

+ FEVER + ALTERED MENTAL STATE

52
Q

What is the cause of DIC

A

sudden increase in exposure to tissue factor (due to sepsis, tumour, pancreatitis, pregnancy, trauma)

Causes activation of coagulation cascade > clotting

body attempts to reverse the clotting via normal anti clot mechanism

causes massive consumption of all clotting factors

53
Q

How does DIC present

A

in VERY UNWELL patient

Everything is wrong: high PT, APTT, low platelets

54
Q

EXPLAIN aetiology of DIC

A

Increase in tissue factor

Causes activation of coagulation cascade > clotting

body attempts to reverse the clotting via normal anti clot mechanism

causes massive consumption of all clotting factors

55
Q

what does ITP stand for

A

Immune / Idiopathic Thrombocytopenic Purpura

56
Q

What is the function of desmopressin in haemophilia/VWD

A

stimulates release of F8 and vWF

57
Q

How do you differentiate ineffective erythropoiesis from red cell aplasia on blood test

A

LOW RETICULOCYTES in red cell aplasia

HIGH RETICULOCYTES in ineffective erythropoesis

58
Q

What should you suspect if reticulocytes are low?

A

Red cell anaemia > parvovirus B19 OR Diamond Blackfan

59
Q

What investigations should you get if low reticulocytes

A

Parvovirus serology

BM aspirate

60
Q

What is sideroblastic anaemia

A

Bone marrow produces ringed sideroblasts instead of healthy RBCs

This causes a microcytic anaemia

61
Q

Why does sideroblastic anaemia occur

A

Body has iron but cannot incorporate it properly into Hb

62
Q

What do sideroblasts look like

A

nucleated erythrocytes (precursors) with iron granules surrounding nucleus

63
Q

What are causes of sideroblastic anaemia

A

CONGENITAL
- genetic

AQUIRED:

  • Myelodysplastic syndrome
  • AML
  • Alcohol use
  • B6 deficiency
  • Lead poisoning, copper poisoning
64
Q

What do you see on blood film for sideroblastic anaemia

A
  • basophillic stippling

- target cells

65
Q

What THREE TYPES of anaemia can you categorise

A

Microcytic (MCV<80)

Normocytic (MCV 80-100)

Microcytic (>100)

66
Q

What are causes of MICROCYTIC anaemia

A

Iron deficiency
Thalassaemia
Anaemia of chronic disease
Sideroblastic anaemia

67
Q

How can you further subdivide NORMOCYTIC anaemia

A

LOW reticulocytes

NORMAL /HIGH reticulocytes

68
Q

What are causes of NORMOCYTIC anaemia with LOW reticulocytes

A

Aplastic anaemia (Fanconi, Schwachen-DIamond)
Red cell aplasia (Parvovirus 19, Diamond-Blackfan)
Malignancy
Renal disease

69
Q

What are causes of NORMOCYTIC anaemia with NORMAL reticulocytes (AKA HAEMOLYTIC CAUSES)

A

INTRINSIC CAUSES

  • Sickle cell
  • Membrane defect (spherocytosis, elliptocyt, paroxysmal nocturnal haemoglobinuria)
  • Enzyme deficiency (G6PD, PK deficiency)
  • immune (ABO/RhD incompatibility, warm/cold AIHA)

EXTRINSIC CAUSES

  • Infection e.g. Malaria
  • MAHA
70
Q

What are causes of MACROCYTIC anaemia

A

MEGALOBLASTIC
- B12/ Folate deficiency

NON-MEGALOBLASTIC

  • liver diserase
  • alcohol
  • drugs
71
Q

Explain beta thalassamia major

A

MOST SEVERE FORM

NO HbA - due to NO functioning beta-globin gene

72
Q

How do you manage beta thalassamia major

A

FATAL without regular blood transfusions

Give BLOOD TRANSFUSIONS + IRON CHELçATION

BONE MARROW TRANSPLANT IS CURE

73
Q

What is HYDROPS Fetalis

A

oedema in min 2 fluid compartments + ascites

74
Q

What are causes for Hydrops

A 
foetal anaemia (iron deficiency, parvovirus B19( 
Maternal infection (CMV, syphilis9 
TTTS
75
Q

How do you treat Hydrops foetal is

A

EXCHANGE TRANSFUSION

76
Q

What is the whole spectrum of disease caused by Parvovirus B19

A

FIFTH DISEASE OR erythema infectiosum OR “slapped cheek syndrome”.

Prodrome of headache, fever, nausea
Bright red rash on cheeks (periorbital pallor)
Rash can stretch to trunk and extremities

77
Q

what are examples of iron chelation regimens available for beta thalassaemia

A

SC desferrioxamine

Oral deferasirox

78
Q

How do you manage DIC

A
  1. Treat underlying cause (usually sepsis)
  2. Supportive care
    3, Replacement therapy (platelet transfusion for platelets, FFP for coag factors, cryoprecipitate transfusions)

Consider Protein C concentrate esp in purpura fulminans

79
Q

How do you manage hereditary spherocytosis

A

supportive care, RBC transufion
Folic acid supplement

Consider splenectomy + encapsulated bacteria vaccine
cholecystectomy

80
Q

What prophylaxis do you give for sickle cell

A

immunise against encapsulated organisms
Daily oral penicillin
Daily oral folic acid
Avoid triggers

81
Q

How do you treat acute crisis of SCD

A 
Oral, IV analgesia 
Good hydration 
Antibiotics for infection 
Oxygen if reduced sats 
Exchange transfusion
82
Q

What management can you consider for chronic problems in sickle cell

A
  • hydroxycarbamide (if recurrent admissions)
  • splemnectomy + imms against encapsulated bacteria
  • BM transplant if sevre

Y5 Paediatrics (30 decks)

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