Paediatric immunology Flashcards

1
Q

Explain the difference between cows milk protein allergy and cows mil intolerance

A
  • Allergy : hypersensitivity to cow’s milk. can be IgE mediated where there is a rapid reaction or non-IgE mediated where rthe reaction occurs over several days
  • Intolerance : nit an allergic process and does not involve the immune system
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2
Q

what GI symptoms are seen in both cow’s milk allergy and intolerance

A

Bloating and wind
Abdominal pain
Diarrhoea
Vomiting

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3
Q

What additional symptoms are seen in cow’s milk protein allergy

A

Urticarial rash (hives)
Angio-oedema (facial swelling)
Cough or wheeze
Sneezing
Watery eyes
Eczema
Rarelu : anaphylaxis

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4
Q

If not clinical, what can be done to support the diagnosis of CMPA

A

Skin prick testing

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5
Q

How is CMPA managed ?

A
  • Breast feeding mothers should avoid dairy products
  • Replace formula with special hydrolysed formulas
  • Severe cases : elemental formulas of basic amino acids
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6
Q

Explain a type 1 hypersensitivity reaction

A
  • IgE mediated
  • Immediate reaction
  • E.g. Food allergies
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7
Q

Explain a type 2 hypersensitivity reaction

A
  • IgG and IgM mediated
  • E.g. Haemolytic disease of the newborn and transfusion reactions
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8
Q

Explain a type 3 hypersensitivity reaction

A
  • Immune complexes cause damage to local tissues
  • E.g. SLE, RA and HSP
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9
Q

Expain a type 4 hypersensitivity reaction

A
  • Caused by T lymphocytes
  • E.g. Organ transplant rejection, contact dermatitis
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10
Q

Give two commonly used tests for diagnosing allergies

A
  • Skin prick
  • Radioallergosorbent (RAST) : determines amount of IgE that reacts to suspected allergies
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11
Q

What allergy testing is useful for contact dermatitis ?

A

Skin patch testing

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12
Q

what causes anaphylaxis ?

A
  • Severe type 1 hypersensitibty reaction
  • IgE mediated = mast cells degranulation
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13
Q

What is the immediate management of anaphylaxis ?

A
  • > IM adrenalin, repeated after 5 minutes if required
  • > Antihistamines, such as oral chlorphenamine or cetirizine
  • > Steroids, usually IV hydrocortisone
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14
Q

How is anaphylaxis confirmed ?

A
  • Measuring serum mast cell tryptase within 6 hours of the event
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15
Q

Explain the adrenaline dose for anaphylaxis based on age

A
  • <6mnths : 100-150micrograms
  • 6mnths - 6 yrs : 150
  • 6-12 yrs : 300
  • Adult & child >12 yrs : 500
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16
Q

what is the best site of IM adrenaline injection

A

Anterolateral aspect of the middle third of the thigh.

17
Q

what causes allergic rhinitis ?

A
  • IgE mediated type 1 hypersensitivity reaction to environmental allergens in the nasal mucosa
18
Q

How does allergic rhinitis present ?

A
  • Runny, blocked and itchy nose
  • Sneezing
  • Itchy, red and swollen eyes
19
Q

How can allergic rhinitis be managed ?

A
  • Avoid trigger
  • Oral antihistamines
  • Nasal corticosteroid spray
20
Q

Explain the different types of antihistamine

A
  • Non sedating : cetirizine, loratadine, fexofenadine
  • Sedating : chlorphenamine (piriton), promethazine
21
Q

What is SCID

A
  • Severe condition causing immunodeficiency in children
  • Results in absent or dysfunctioning T and B cells
22
Q

what causes most cases on SCID

A
  • Mutations in the common gamma chain on the X chromosome that codes for interleukin receptors on T and cells
  • X linked recessive
23
Q

How does SCID present ?

A

First few mnths of life with :

  • Persistent severe diarrhoea
  • Failure to thrive
  • Opportunistic infections that are more frequent or severe than in healthy children
  • Unwell after live vaccinations such as the BCG, MMR and nasal flu vaccine
24
Q

what is a rare cause of SCID

A
  • Omenn syndrome
25
Q

What are the classic features of Ommen syndrome ?

A
  • A red, scaly, dry rash (erythroderma)
    -Alopecia
  • Diarrhoea
  • Failure to thrive
  • Lymphadenopathy
  • Hepatosplenomegaly
26
Q

What is an example of a cause of mild immunodeficiency

A
  • Selective immunoglobulin A deficiency
  • Pts have a tendency towards recurrent mucous membrane infections (e.g. LRTI, autoimmune conditions).
27
Q

Name 3 B cell or immunoglobulin disorders

A
  • Selective immunoglobulin A deficiency
  • Common variable immunodeficiency
  • X-linked Agammaglobulinaemia
28
Q

Name 5 T cell disorders

A
  • DiGeorge syndrome
  • Purine Nucleoside phosphorylase deficiency
  • Wiskott-Aldrich syndrome
  • Ataxic Telangiectasia
29
Q

What causes DiGeorge syndrome

A
  • Microdeletion in a portion of chromosome 22
  • Incomplete development of thymus gland -> inability to create functional T cells
30
Q

What are the features of DiGeorge syndrome

A

CATCH-22

  • C – Congenital heart disease
  • A – Abnormal facies (characteristic facial appearance)
  • T – Thymus gland incompletely developed
  • C – Cleft palate
  • H – Hypoparathyroidism and resulting Hypocalcaemia
  • 22nd chromosome affected
31
Q

what is purine nucleoside phosphorylase deficiency ?

A
  • Autosomal recessive condition causing a deficiency in PNPase
  • Without it, build up of dGTP = toxic to T lymphocytes
32
Q

What is Wiskott-Aldrich syndrome ?

A
  • X linked recessive condition with a mutation on WAS gene = abnormal functioning T cells
33
Q

What are the features of WAS

A
  • Thrombocytopenia
  • Immunodeficiency
  • Neutropenia
  • Eczema
  • Recurrent infections
  • Chronic bloody diarrhoea
34
Q

What is Ataxic Telangiectasia ?

A
  • Autosomal recessive condition affecting the gene coding for the ATM serine / threonine kinase on chromosome 11
35
Q

What are the features of ataxic telangiectasia ?

A
  • Low numbers of T-cells and immunoglobulins, causing immunodeficiency and recurrent infections.
  • Ataxia
  • Telangiectasia, particularly in the sclera and damaged areas of skin
  • Predisposition to cancers, particularly haematological cancers
  • Slow growth and delayed puberty
  • Accelerated ageing
  • Liver failure
36
Q

Name 2 complement disorders

A
  • Hereditary angioedema
  • Mannose-Binding Lectin Deficiency
37
Q

A patient presents with episodes of unexplained lip swelling (angioedema). What test should you perfrom and what is the likely underlying diagnosis ?

A
  • C4 levels
  • If C1 esterase inhibitor deficiency (hereditary angioedema) is the underlying cause, the C4 levels will be low.