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Paeds > Haem > Flashcards

Haem Flashcards

1
Q

At birth what is level of Hb? Why is this?

A

14-21.5 g/dl

to compensate for low O2

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2
Q

What are the differences between babies born at term and prem in terms of Hb?

A

Stores of iron, folic acid and b12 in term and prem are adequate at birth

however after 2-4 months - prems will run out and need supplements

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3
Q

How would you define anemia in terms of haemoglobin levels?

A

Neonate - <14g/dL

1-12 months : <10g/dL

1-12 years : <11g/dL

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4
Q

What are the 3 mechanisms that cause anaemia?

A

Reduced cell production (ineffective erythropoiesis and red cell aplasia)

Increased red cell destruction

Blood Loss

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5
Q

What are some causes of impaired red cell production due to red cell aplasia?

A

Parvovirus

Diamond-Blacktan anaemia

Transient erythroblastopenia

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6
Q

What are some causes of impaired red cell production due to ineffective erythropoiesis?

A

iron deficiency

folic acid efficiency

chronic inflamm (JIA)

chronic renal failure

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7
Q

What are some causes of increased red cell destruction?

A

hereditary spherocytosis

G6PD deficiency

Thalassaemias, Sickle-cell

haemolytic disease of newborn

autoimmune haemolytic anaemia

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8
Q

What are some causes of blood loss?

A

fetomaternal bleeding

meckels diverticulum

von Willebrand’s

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9
Q

What are some diagnostic clues to ineffective erythropoiesis?

A

Normal reticulocyte count

abnorm MCV of red cells

i) low in iron deficiency
ii) increased in folic acid deficiency

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10
Q

What are some causes of iron deficiency?

A

inadequate intake

malabsorption

blood loss

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11
Q

What are some sources of iron?

A

breast milk

infant formula

cows milk

red meat

liver

oily fish

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12
Q

What some features of iron deficiency anaemia?

A

asymptomatic until <6-7g/dL

  • then children tire more easily and feed more slowly than usual
  • ‘pica’ - eating non-food
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13
Q

What is diagnostic of Red Cell Aplasia?

A

Low reticulocytes despite low Hb

Normal bilirubin

-ve Coombs test

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14
Q

How would haemolytic anaemia present?

A

Anaemia

Hepatomegaly

Splenomegaly

Increased blood levels of unconjugated bilirubin

Excess urinary urobilinogen

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15
Q

What is diagnostic of haemolytic anaemia?

A

increased reticulocytes

polychromasia - reticulocytes are lilac

spherocytes

positive coombs

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16
Q

How does sickle-cell present?

A

moderate anaemia

increased susceptibility to infection

osteomyelitis

vaso-occlusive crises

haemolytic crises

aplastic crises

sequestration crises

priapism

splenomegaly

17
Q

What are long-term consequences of sickle cell

A

short

delayed puberty

stroke

cardiac enlargement

HF

renal dysfunction

leg ulcers

18
Q

How would you manage sickle-cell?

A

prophylaxis - pral penicillin
oral folic acid

avoid cold, dehydration, excessive exercise

19
Q

How would beta thalassaemia present?

A

severe anaemia from 3-6 months

jaundice

failure to thrive

extramedullary haemopoiesis

classical facies - maxillary overgrowth and skull bossing

20
Q

When does the classical facies in beta -thalassaemia occure?

A

occurs without regular blood transfusions

21
Q

What is Fanconi anaemia? What does it cause?

A

AR condition

short stature

abnormal radii and thumbs

renal malform

pigmented skin legions

22
Q

What is the risk associated with Fanconi anaemia?

A

Death from bone marrow failure

transformation to acute leukaemia

23
Q

What inheritance is haemophilia?

A

X-linked recessive

24
Q

What are the deficiencies in haemophilia a and b?

A

haemophilia A - F VIII deficiency

haemophilia B - F IX deficiency

25
Q

How would you grade haemophilia

A

severe - spontaneous joint/muscle bleeds

moderate - bleed after minor trauma

mild - bleed after surgery

26
Q

How would you manage haemophilia? What are some complications associated with the treatment?

A

recombinant F VIII/IX - intraV

complic - antibodies to FVIII/FIX, cause decrease or inhibit treatment > need a higher dose

vascular access difficult with risk of thrombosis

27
Q

What is the function of von Willebrand factor?

A

facilitates platelet adhesion to damaged endothelium

carrier protein for FVIII:C

28
Q

How does vWd present?

A

bruising,

excessive prolonged bleeding after surgery

epistaxis

menorrhagia

29
Q

How would you treat vWd?

A

DDAVP (desmopressin)

Plasma derived FVIII concentrate

30
Q

What is immune thrombocytopenia?

A

commonest thrombocytopenia

destruction of circulating platelets by antiplatelet IgG > decreased platelets

31
Q

How would immune thrombocytopenia present?

A

2-10 yrs old

1-2 weeks after a viral infection

petechiae, purpura and/or superficial bruising

epistaxis

32
Q

How does immune thrombocytopenia normally resolve?

A

self-limting

if complications > oral prednisolone, IV anti-D, immunoglobulin

33
Q

What is treatment for chronic ITP?

A

rituximab

splenectomy

screen for SLE

Paeds (13 decks)

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