Paeds IMMUNITY

Question 1

What are the general signs and symptoms of T cell defects?

Answer 1

Severe viral + fungal infections

Question 2

What are the general signs and symptoms of B cell defects?

Answer 2

Severe bacterial infections

Question 3

What are the general signs and symptoms of neutrophil defects?

Answer 3

Recurrent bacterial infections + invasive fungal infections

Question 4

What are the general signs and symptoms of NK cell defects?

Answer 4

Recurrent viral infections

Question 5

Give six examples of T cell defects

Answer 5

SCID
HIV
Ataxia telangiectasia
DiGeorge syndrome
Wiskott-Aldrick syndrome
Duncan disease

Question 6

Give four examples of B cell defects

Answer 6

Bruton’s agammaglobulinaemia
CVID
Hyper IgM
IgA deficiency

Question 7

Given an example of a neutrophil defect disease

Answer 7

Chronic granulomatous disease

Question 8

Give two examples of NK defects

Answer 8

Classical + functional NK cell deficiency

Question 9

What are the 2 main signs and symptoms of leukocyte adhesion deficiency?

Answer 9

Delayed separation of umbilical cord
Chronic skin ulcers

Question 10

What are the 2 main signs and symptoms of complement defects?

Answer 10

Recurrent bacterial infections (esp. encapsulated bacteria) + SLE-like illness

Question 11

What is hyper-IgE also known as?

Answer 11

Job/Buckley syndrome

Question 12

What are the signs and symptoms of hyper-IgE?

Answer 12

Eczema
Coarse facial features
Recurrent RTIs
Cold abscesses
Candidiasis

Question 13

What is the pathophysiology (briefly) of ataxia telangiectasia?

Answer 13

Defective DNA repair causing T cell defect

Question 14

What are people with ataxia telangiectasia at increased risk of?

Answer 14

Lymphoma

Question 15

What are the signs and symptoms of ataxia telangiectasia?

Answer 15

Cerebellar ataxia
Developmental delay
Telangiectasia in eyes

Question 16

What is the inheritance pattern of Wiskott-Aldrich syndrome?

Answer 16

X-linked

Question 17

At around what age does Wiskott-Aldrich syndrome present?

Answer 17

7 months

Question 18

What are the signs and symptoms of Wiskott-Aldrich syndrome?

Answer 18

WATER:
Wiskott-Aldrich
Thrombocytopaenia
Eczema
Recurrent infections

Question 19

How can Wiskott-Aldrich syndrome be differentiated from ITP?

Answer 19

WAS presents ~7 months but ITP is more like 4 years

Question 20

What is the cause of eczema in Wiskott-Aldrich syndrome?

Answer 20

Raised IgA + IgE

Question 21

What is the cause of recurrent infection in Wiskott-Aldrich syndrome?

Answer 21

Low IgG + IgM

Question 22

How is Wiskott-Aldrich syndrome managed?

Answer 22

IVIg –> HSCT

Question 23

What is the inheritance pattern of Duncan disease?

Answer 23

X-linked

Question 24

What is the pathophysiology of duncan disease?

Answer 24

Inability to generate a normal response to EBV

Question 25

What are the signs and symptoms of duncan disease?

Answer 25

Death in initial EBV or development of a secondary B cell lymphoma

Question 26

How does a non-IgE mediated allergy present?

Answer 26

Erythema Atopic eczema GORD Change in frequency of stools Blood/mucus in stools Constipation Food aversion

Question 27

What should an allergy-focused history contain?

Answer 27

Classification (speed, onset, severity, reproducability) Atopic hx (personal or FH) Food diary Details of food avoidance + why Details of any feeding hx (age of weaning etc) Cultural/ religious factors Any previous elimination trials

Question 28

What are the 2 tests that can be done for allergy?

Answer 28

Test 1 = skin prick test Test 2 = measurement of specific IgE antibodies

Question 29

When would you refer to a specialist for allergy?

Answer 29

Faltering growth Severe atopic eczema Multiple allergies, persisting suspicion, hx of an acute systemic/ severe delayed reaction

Question 30

How should allergy be managed?

Answer 30

Specialist care if indicated Avoid relevant foods MDT: advice from paediatric dietician to avoid nutritional deficiencies Teach family + child how to manage allergic attack Written info + adequate training

Question 31

What are the classifications of allergic rhinitis?

Answer 31

Intermittent vs persistent Mild vs severe Seasonal vs perennial

Question 32

What other differentials need to be ruled out in suspected allergic rhinitis?

Answer 32

Nasal polyps Deviated nasal septum Mucosal swelling/ depressed + widened nasal bridge

Question 33

How is occasional symptomatic relief achieved in allergic rhinitis?

Answer 33

Any age: intranasal azelastine (antihistamine) 2-5 y/o: oral certirizine

Question 34

How is frequent symptomatic relief achieved in allergic rhinitis?

Answer 34

If main issue nasal blockage/ polyps: intranasal beclomethasone If main issue sneezing/ discharge: intranasal CS or oral antihistamine

Question 35

What is SCIT?

Answer 35

Subcutaneous Immunotherapy Used to administer specific allergen immunotherapy SC injection on a regular basis for 3-5 years Can provide protection for any years but has risk of inducing anaphylaxis

Question 36

Who is most likely to develop a cow's milk protein allergy?

Answer 36

Formula-fed children

Question 37

How should cow's milk protein allergy be investigated?

Answer 37

Same as 'food allergy'

Question 38

How should cow's milk protein allergy be managed?

Answer 38

Trial cow's milk elimination diet for 2-6 weeks: In breast-fed babies, mother should exclude cow's milk protein from her diet Consider prescribing daily 1g calcium + 10mcg vit D In formula-fed babies, replace cows milk based formula with hypoallergenic formula

Question 39

What is the most common complication of measles?

Answer 39

Acute otitis media

Question 40

Describe the presentation of meconium ileus

Answer 40

Large volumes of billious vomiting