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Paediatrics RACP Exam Study > Paediatric Immunology 2 > Flashcards

Paediatric Immunology 2 Flashcards

1
Q

How might you treat CGD?

A
  • Cotrimoxazole prophylaxis (decreases serious infections from annually to Q3.5y)
  • Itraconazole prophylaxis
  • IFN-gamma
  • BMT – depending on severity of clinical phenotype
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2
Q

How does neutropenia present clinically?

A
  • Fever
  • Aphthous stomatitis
  • Gingivitis
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3
Q

Deficiency of which complement component is most associated with severe pyogenic infections?

A

C3 - common final pathway

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4
Q

Which cells are implicated in allergic disease?

A
  • Th2 T cells

- Eosinophils

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5
Q

Which cytokine drives eosinophil production?

A

IL-5

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6
Q

Which cytokine has a role in inducing a generalised anti-viral state of cellular metabolism in other cells?

A

Interferon 1 (alpha and beta)

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7
Q

What does alpha-1 antitrypsin do?

A

Prevents tissue destruction

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8
Q

What diseases are associated with NALP3 mutations?

A
  • Familial cold urticaria
  • Muckle-Wells
  • Neonatal onset multisystem inflammatory disease
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9
Q

It is important to avoid which medication in hereditary angioedema?

A

ACE inhibitors

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10
Q

What does exulizumab do?

A

Monoclonal C5a antibody, useful for PNH and aHUS

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11
Q

What is a Type 1 reaction?

A

Immediate IgE mediated reaction e.g. anaphylxis, urticaria, angioedema, anaphylactic shock, bronchial asthma, rhinitis, conjunctivitis

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12
Q

What is a Type 2 hypersensitivity reaction?

A

Antibody mediated cytotoxic reaction e.g. immune haemolytic anaemia

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13
Q

What types of allergy do you do intradermal testing for?

A

Drug allergy

Insect venom allergy

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14
Q

What is a Type 3 hypersensitivity reaction?

A

Immune complex mediated e.g. serum sickness, vasculitis

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15
Q

When is tryptase highest?

A

Peaks 60-90mins after anaphylaxis, can be taken 15mins after, helpful up to 3 hrs but may be high for up to 5hrs

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16
Q

What autoantibodies are positive in polyarteritis nodosa?

A

None - trick question!

Associated with strep infection, Hep B, chronic hepatitis

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17
Q

What does AIRE actually do?

A

Induces expression of self-proteins in thymic medullary cells
Regulates central tolerance

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18
Q

How does toxic shock syndrome happen?

A

Bacteria produce a superantigen toxin that allows the nonspecific binding of MHC II with T-cell receptors, resulting in polyclonal T-cell activation

19
Q

MHC class I pathway is for…?

A
  • Sampling and processing proteins from the cytoplasm of somatic non APC cells
  • Therefore if intracellular organisms invade cells and cause upregulation/production of unusual stuff, cytotoxic T cells (CD8) can kill it
  • If the intracellular organism downregulates MHC then NK cells can kill it
20
Q

What type of reaction is Steven-Johnson Syndrome?

A
  • Type IV hypersensitivity
  • drug or its metabolite stimulates cytotoxic T cells (i.e. CD8+ T cells) and T helper cells (i.e. CD4+ T cells) to initiate autoimmune reactions that attack self tissues
21
Q

What is the difference between SJS and TEN?

A
  • TEN more severe

- >30% of body

22
Q

Why don’t B cells develop in Bruton’s (XLA)?

A

Because there is no bruton tyrosine kinase i.e. no SIGNALLING (critical signalling molecule)

23
Q

What would you expect from a TH2 response?

A

High IgE
High eosinophils
Raised IL4
Raised IgG4

24
Q

Diseases with inflammasome reaction respond to blockade of….

A

IL-1 which is anakinra

25
Q

Monoclonal antibody to IL4 targets which T cells…?

A

TH2

26
Q

Eosinophilic granulomatosis with polyangiitis (EGPA or Churg-Strauss syndrome) is characterized by….

A

Asthma, eosinophilia, and systemic vasculitis of multiple organs

27
Q

The anaphylotoxins are….

A

C3a, C4a, and C5a

28
Q

Cyclosporin targets…

A
  • T-cells
  • Binds to the cytosolic protein cyclophilin (aka immunophilin) -> inhibits calcineurin -> less transcription of interleukin 2 -> T Cells not activated
29
Q

Type 1 Interferons are associated with which sort of infective organism?

A

Viruses

e.g. IFN-α and IFN-β

30
Q

What drugs do we avoid in C1 deficiency?

A

ACEI

Oestrogen

31
Q

Acute renal transplant rejection is mediated by…

A

Direct recognition of foreign MHC (HLA) by T cells

32
Q

Exact HLA match probabiity between siblings…

A

25%

33
Q

Which interleukin is needed for T-cell development?

A

IL-2

34
Q

CRP production is stimulated by which cytokine?

A

IL-6

35
Q

What are the laboratory and imaging findings associated with ataxia-telangiectasia?

A
  • High AFP
  • low Ig
  • white blood cell chromosome fragility

MRI would show cerebellar volume loss, and compensatory enlargement of the fourth ventricle

36
Q

What % of children outgrow peanut allergy?

A

20%-25%

37
Q

SJS/TEN is which type of immune reaction?

A

Type IV

38
Q

Serum sickness is which type of immune reaction?

A

Type III

39
Q

What is the treatment for anaphylaxis?

A

IM - adrenaline 0.01 ml/kg of 1:1000

IV - adrenaline 0.01 ml/kg of 1:10000 or 0.01mg/kg

40
Q

Increased allergy is associated with…

A
  • Decreased allergen and microbial diversity exposure

- Excessive use of antibiotics in first 2 years of life can change gut flora and therefore modulate allergy

41
Q

You can help prevent atopic dermatitis by…

A
  • Taking probiotics during pregnancy

- Applying bland moisturizer in infancy

42
Q

What are the complement fixing antibodies?

A
  • IgG and IgM
43
Q

HOT T Bone sTEAk acronym stands for…?

A

Interleukin functions

  • IL-1 fever
  • IL-2 for T cell stimulation
  • IL-3: Bone marrow stimulator (sim to GM-CSF)
  • IL-4: IgE (class switching from IgG), B cell growth
  • IL-5: IgA, eosinophils

Paediatrics RACP Exam Study (30 decks)

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