Paediatric Haematology

Question 1

What is kernicterus?

Answer 1

This is when free bilirubin crosses the blood-brain barrier and deposits in the basal ganglia

Question 2

A child presents with sudden onset of purpura and petechiae and the child is otherwise well despite having had a viral illness a few weeks ago. What is the most likely diagnosis?

Answer 2

Idiopathic Thrombocytic purpura

Question 3

What will be seen on a blood count in ITP

Answer 3

Low platelet count with normal white and red blood cells

Question 4

What must be ruled out in ITP?

Answer 4

Leukaemia

Take a bone marrow aspirate prior to starting any treatments for ITP

Question 5

What is the most common cause of haemolysis in children?

Answer 5

Hereditary spherocytosis

Question 6

What mode of inheritance is hereditary spherocytosis?

Answer 6

Autosomal Dominant

Question 7

What viral infection can cause transient severe anaemia?

Answer 7

Parvovirus B19

Question 8

What is the most common acquired bleeding disorder in children?

Answer 8

Idiopathic Thrombocytic Purpura

Question 9

What is Henoch-Schonlein Purpura?

Answer 9

An acute immune complex mediated vasculitis.

Question 10

What is the common triad of symptoms in HSP?

Answer 10

Purpura
Arthritis / arthralgia
Abdominal pain

Question 11

Explain the physiology of rhesus haemolytic disease:

Answer 11

A rhesus -ve mother will deliver a rhesus +ve baby. The leak of foetal blood into the mothers circulation will cause her to mount an immune response of anti-D IgG antibodies.
This will then affect any subsequent pregnancies with rhesus +ve babies.

Question 12

What is the clinical consequence of rhesus disease?

Answer 12

The IgG antibodies will cause haemolysis of the red blood cells in the foetus, causing anaemia. This will then lead to the development of hydrops fetalis

Question 13

What is hydrops fetalis?

Answer 13

This is the abnormal accumulation of fluid in two or more compartments e.g. ascites, pericardial effusions etc which carries a high rate of mortality.

Question 14

How is rhesus haemolytic disease prevented?

Answer 14

All pregnant women who are rhesus -ve are given a rhesus D antibody vaccine at 28 weeks gestation

Question 15

When is newborn jaundice concerning?

Answer 15

Within the first 24 hours or after 2 weeks

Question 16

An otherwise apparently healthy 3 week old newborn presents with prolonged jaundice, clay coloured stools and very yellow urine. Parents are concerned because they can feel a “mass” in the babies tummy. What is the likely diagnosis?

Answer 16

Biliary atresia

The parents can feel the spleen / liver

Question 17

What is the treatment for biliary atresia?

Answer 17

If it is caught within the first 6 weeks of life the Kasai procedure is done to improve biliary drainage. Past 6 weeks the child will be in acute liver failure and will require a transplant.

Question 18

What is the Kasai procedure?

Answer 18

A hepatoportoenterostomy
This is where the extrahepatic biliary tree is identified and an intestinal limb is attached to the porta hepatis to allow for bile drainage.

Question 19

What is the pathology of biliary atresia?

Answer 19

There is an absence of intra- or extrahepatic bile ducts which leads to a conjugated hyperbilirubinemia causing obstructive jaundice. If untreated it will lead to liver failure and become fatal.

Question 20

Mild physiological jaundice is common in premature newborns, true or false?

Answer 20

True

Due to liver immaturity

Question 21

What group of (term) babies are more prone to developing jaundice?

Answer 21

Breastfed babies

Question 22

What should be measured to exclude conjugated hyperbilirubinemia in suspected breastfed jaundice?

Answer 22

Split bilirubin

Question 23

What chronic illness can lead to cholestasis in the newborn?

Answer 23

cystic fibrosis

Question 24

How is biliary atresia diagnosed?

Answer 24

Rising conjugated bilirubin levels

Diagnostic isotope scan

Question 25

A deficiency of _______ can lead to hemorrhagic disease of the newborn, causing abnormal bruising and bleeding?

Answer 25

Vitamin K

Question 26

What is the most common childhood malignancy?

Answer 26

Leukaemia

Question 27

What is the peak age for acute lymphoblastic leukaemia?

Answer 27

2-5 years old

Question 28

What are some of the symptoms of ALL?

Answer 28

Symptoms of pancytopenia - pallor, infection and bleeding
Fatigue
Anorexia
Malaise
Lymphadenopathy / painless lumps in armpit, neck and groin

Question 29

What is tumour lysis syndrome?

Answer 29

This is when the breakdown of large numbers of malignant cells leads to very high serum urate, phosphate and potassium levels.
Urate crystals can cause kidney failure,

Question 30

How should febrile neutropenia be managed?

Answer 30

Significant fever or signs of infection in a neutropenic patient should be investigated thoroughly and treated with aggressive broad spectrum antibiotic therapy.

Question 31

What is the best way to prevent tumour lysis syndrome?

Answer 31

Good hydration

use of allopurinol

Question 32

What should immunosuppressed children not be given as they undergo treatment for leukaemia (or other cancer) and what should be given if they have contact with varicella virus?

Answer 32

Live vaccines

Give a specific immunoglobulin. If they develop varicella treat with aciclovir

Question 33

What is the 5 year survival rate for ALL in children who have appropriate treatments?

Answer 33

80-95%