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Flashcards in Haematological Disorders Deck (139)
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1

When is HbF very low in healthy children - when is it not?

By age 1
Increased proportions of HbF are indicators of severe inherited disorders of haemoglobin production - haemoglobinopathies

2

Hb at birth

14-21.5g/dl to compensate for low oxygen concentration in fetus

3

What happens to Hb after birth

Falls over first few weeks of life, mainly due to reduced red cell production, to 10g/dl at 2 months of age

4

What happens to Hb after birth in pre-term babies?

It has a steaper fall to a mean of 6.5-9g/dl at 4-8 weeks chronological age

5

What are the iron, B12 and folic acid stores like in term and preterm infants at birth and after birth?

Iron, B12 and folic acid are adequate at birth in term and preterm babies
However in preterm babies stores of iron and folic acid are lower and are depleted more quickly leading to deficiency after 2-4months if recommended daily intakes are not maintained by supplements

6

Anaemia value in neonate

Hb less than 14g/dl

7

Anaemia value in 1-12months old

Hb less than 10g/dl

8

Anaemia value in 1-12 years

Hb less than 11g/dl

9

What is red cell aplasia

Complete absence of red cell production

10

What is ineffective erythropoeisis?

Red cell production is normal/increased rate but differentiation or survival of red cells is defective

11

What are the main causes of iron deficiency anaemia x3

Inadequate intake (common in infants)
Malabsorption
Blood loss

12

Which milk is not good for maintaining infant iron levels?

Cows milk because it has a higher iron content than breast milk but only 10% of the iron is absorbed
Therefore infants should not be fed unmodified cows milk

13

At what Hb level do children become symptomatic with anaemia?

6-7g/dl

14

How do children with iron deficiency anaemia present?

Pica- eating non-food materials such as soil, chalk, gravel or foam rubber

15

What are indicators on blood tests of iron deficiency anaemia

Microcytic, hypochromic anaemia (low MCV and MCH)
Low serum ferritin

16

Management of iron deficiency anaemia in infants?

Increase oral iron intake with supplementation - Sytron or Niferex are best tolerated preparations
Or just increase iron rich foods

17

What are the 3 main causes of red cell aplasia in children?

1) Diamond-Blackfan anaemia - congenital red cell aplasia
2) Transient erythroblastopenia of childhood
3) Parvovirus B19 infection in children with haemolytic anaemia

18

Diagnostic features of red cell aplasia x4

Low reticulocyte count despite normal Hb
Normal bilirubin
Negative direct antiglobulin/Coombs test
Absent red cell precursors on bone marrow examination

19

What is Diamond-Blackfan anaemia?

It is a rare congenital disease of red cell aplasia

20

Inheritance of Diamond-Blackfan anaemia

20% family history - remaining 80% are sporadic mutations
RPS (ribosomal protein) genes implicated in some cases

21

Presentation of Diamond-Blackfan anaemia

Most present at 2-3 months of age but 25% present at birth

22

Features of Diamond-Blackfan anaemia x2

Anaemia
Also congenital abnormalities such as short stature or abnormal thumbs

23

Treatment of Diamond-Blackfan anaemia x2

Oral steroids
Monthly red cell transfusions for children not responsive to steroids

24

What is transient erythoblastopenia of childhood?

Red cell aplasia usually triggered by viral infections
Same haemotological features as D-Blackfan anaemia

25

Prognosis of transient erythroblastopenia of childhood

Always recovers - usually within several weeks (hence differs from d-blackfan)

26

Inheritance of transient erythroblastopenia of childhood

No family history

27

When does haemolysis lead to anaemia?

When the bone marrow can no longer increase red cell production to compensate for the premature destruction of red cells

28

Main causes of haemolytic anaemias in children? What is uncommon children

Intrinsic abnormalities of RBCs (membrane and enzyme disorders and haemoglobinopathies)
Immune haemolysis is uncommon

29

What does haemolysis from increased RBC breakdown lead to? x4

Anaemia
Hepatomegaly and splenomegaly
Increased blood levels of unconjugated bilirubin
Increased urinary urobilinogen

30

Diagnostic clues to haemolytic anaemia x4

Increased reticulocyte count
Unconjugated bilirubinaemia and urinary urobilinogen
Abnormal appearance of red blood cells on film (spherocytes, sickle shaped or very hypochromic)
Increased red blood cell precursors in bone marrow