Child health- Haematological conditions

Question 1

what are issues with anaemia diagnosis in children?

Answer 1

there are many different ranges depending on the age of the child

Question 2

three main mechanisms for anaemia

Answer 2

haemolysis
haemorrhage
reduced haematopoiesis

Question 3

what is megaloblastic anaemia caused by

Answer 3

b12 and folate deficiency- casues failure of the DNA to produce effective RBC

Question 4

what is Fanconi Anaemia

Answer 4

genetic disorder which causes bone marrow failure

Question 5

why should a patient with Fanconi anaemia not be irradiated

Answer 5

genetic mutations which cannot repair DNA- exposure to radiation can easily cause malignancies

Question 6

what causes pica

Answer 6

iron deficiency

Craving and consumption of non food stuff (sand, chalk, crayons, soil)

Question 7

how cows milk protein cause iron deficiency anaemia?

Answer 7

causes GI bleeding!!

Question 8

treatment for iron deficiency?

Answer 8

REPLACEMENT!

oral therapy

Infusions

Question 9

what are the two main causes of haemolysis

Answer 9

intracorpuscular (or intravascular)
extra corpuscular (or extra vascular)

Question 10

what are the types of intracorpuscular haemolysis?

Answer 10

haemoglobins
enzymes
membranopathies

more often congenital

Question 11

what are the types of extra corpuscular haemolysis

Answer 11

auto immune
fragmentation
hypersplenism
plasma factors

more often acquiredh

Question 12

how to screen for haemolysis

Answer 12

FBC
Retic
Blood film
LDH
Haptoglobin
bilirubin

DIRECT COOMBS TEST IS THE DEFINITIVE AUTO IMMUNE HAEMOLYSIS TEST

Question 13

which cells clear RBC in the spleen?

Answer 13

macrophages

Question 14

define G6PD

Answer 14

x linked recessive conditoin due to defieicency of Glucose-6-phosphaste dehydrogenase

Question 15

how does a spherocyte

Question 16

what is sporadic haemolysis in G6PD

Answer 16

induced intravascular haemolysis

Question 17

what induces sporadic haemolysis in G6PD?

Answer 17

drugs

fava beans (fava bean fever!)

fever

acidosis

Question 18

what are symptoms of sporadic haemolysis in G6PD

Answer 18

rigors, fever, back pain

Question 19

how is sporadic haemolysis in g6PD treated?

Answer 19

stopping precipitant (eg medication change)

transfusion

renal support

Question 20

which is the commonest hereditary haemolytic anaemia in europeans

Answer 20

1/5000 have hereditary spherocytosis

Question 21

describe hereditary spherocytosis

Answer 21

typically autosomal dominant, BUT no family Hx in 25% of cases

hetereogenous- deficiencies of spectrin (41.5%), ankyrin (1.5%), band 3 (17%), band 4.2 (21.5%)

Question 22

describe the clinical picture of hereditary spherocytosis

Answer 22

clinical effects vary from mild ot transfusion dependence

tends to be similar within families

offer folic acid, transfuse if needed.

Splenectomy if SEVERE

Question 23

what is the most common form of cancer in children

Answer 23

leukaemias

Question 24

which is the most common form of paediatric leukaemia?

Answer 24

ALL (85%)

2-4 cases per 100,000

classified also as B and T cell

2 years of Tx- >90% positive prognosis

Question 25

how many cases of AML are there a year in the UK

Answer 25

approx 70 cases in 0-16 year olds

Question 26

what are the survival rates for AML

Answer 26

60-70% approx

Question 27

what are the two broad terms for treatment of leukaemia

Answer 27

supportive
- prompt tx of infection
- prevention of tumour lysis
- blood product support

specific
- chemo
- targeted therapy eg Blinatumomab, Inotuzumab
- bone marrow Transplant

Question 28

what can the treatments for leukaemia affect?

Answer 28

psychological function
family and social function
growth
endocrine function
puberty
fertility
intellectual
secondary malignancies