Paeds IMMUNITY Flashcards
What are the general signs and symptoms of T cell defects?
Severe viral + fungal infections
What are the general signs and symptoms of B cell defects?
Severe bacterial infections
What are the general signs and symptoms of neutrophil defects?
Recurrent bacterial infections + invasive fungal infections
What are the general signs and symptoms of NK cell defects?
Recurrent viral infections
Give six examples of T cell defects
SCID
HIV
Ataxia telangiectasia
DiGeorge syndrome
Wiskott-Aldrick syndrome
Duncan disease
Give four examples of B cell defects
Bruton’s agammaglobulinaemia
CVID
Hyper IgM
IgA deficiency
Given an example of a neutrophil defect disease
Chronic granulomatous disease
Give two examples of NK defects
Classical + functional NK cell deficiency
What are the 2 main signs and symptoms of leukocyte adhesion deficiency?
Delayed separation of umbilical cord
Chronic skin ulcers
What are the 2 main signs and symptoms of complement defects?
Recurrent bacterial infections (esp. encapsulated bacteria) + SLE-like illness
What is hyper-IgE also known as?
Job/Buckley syndrome
What are the signs and symptoms of hyper-IgE?
Eczema
Coarse facial features
Recurrent RTIs
Cold abscesses
Candidiasis
What is the pathophysiology (briefly) of ataxia telangiectasia?
Defective DNA repair causing T cell defect
What are people with ataxia telangiectasia at increased risk of?
Lymphoma
What are the signs and symptoms of ataxia telangiectasia?
Cerebellar ataxia
Developmental delay
Telangiectasia in eyes
What is the inheritance pattern of Wiskott-Aldrich syndrome?
X-linked
At around what age does Wiskott-Aldrich syndrome present?
7 months
What are the signs and symptoms of Wiskott-Aldrich syndrome?
WATER:
Wiskott-Aldrich
Thrombocytopaenia
Eczema
Recurrent infections
How can Wiskott-Aldrich syndrome be differentiated from ITP?
WAS presents ~7 months but ITP is more like 4 years
What is the cause of eczema in Wiskott-Aldrich syndrome?
Raised IgA + IgE
What is the cause of recurrent infection in Wiskott-Aldrich syndrome?
Low IgG + IgM
How is Wiskott-Aldrich syndrome managed?
IVIg –> HSCT
What is the inheritance pattern of Duncan disease?
X-linked
What is the pathophysiology of duncan disease?
Inability to generate a normal response to EBV
What are the signs and symptoms of duncan disease?
Death in initial EBV or development of a secondary B cell lymphoma
How does a non-IgE mediated allergy present?
Erythema
Atopic eczema
GORD
Change in frequency of stools
Blood/mucus in stools
Constipation
Food aversion
What should an allergy-focused history contain?
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
What are the 2 tests that can be done for allergy?
Test 1 = skin prick test
Test 2 = measurement of specific IgE antibodies
When would you refer to a specialist for allergy?
Faltering growth
Severe atopic eczema
Multiple allergies, persisting suspicion, hx of an acute systemic/ severe delayed reaction
How should allergy be managed?
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
What are the classifications of allergic rhinitis?
Intermittent vs persistent
Mild vs severe
Seasonal vs perennial
What other differentials need to be ruled out in suspected allergic rhinitis?
Nasal polyps
Deviated nasal septum
Mucosal swelling/ depressed + widened nasal bridge
How is occasional symptomatic relief achieved in allergic rhinitis?
Any age: intranasal azelastine (antihistamine)
2-5 y/o: oral certirizine
How is frequent symptomatic relief achieved in allergic rhinitis?
If main issue nasal blockage/ polyps: intranasal beclomethasone
If main issue sneezing/ discharge: intranasal CS or oral antihistamine
What is SCIT?
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
Who is most likely to develop a cow’s milk protein allergy?
Formula-fed children
How should cow’s milk protein allergy be investigated?
Same as ‘food allergy’
How should cow’s milk protein allergy be managed?
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
What is the most common complication of measles?
Acute otitis media
Describe the presentation of meconium ileus
Large volumes of billious vomiting
