Paediatric Immunology 2 Flashcards
(43 cards)
How might you treat CGD?
- Cotrimoxazole prophylaxis (decreases serious infections from annually to Q3.5y)
- Itraconazole prophylaxis
- IFN-gamma
- BMT – depending on severity of clinical phenotype
How does neutropenia present clinically?
- Fever
- Aphthous stomatitis
- Gingivitis
Deficiency of which complement component is most associated with severe pyogenic infections?
C3 - common final pathway
Which cells are implicated in allergic disease?
- Th2 T cells
- Eosinophils
Which cytokine drives eosinophil production?
IL-5
Which cytokine has a role in inducing a generalised anti-viral state of cellular metabolism in other cells?
Interferon 1 (alpha and beta)
What does alpha-1 antitrypsin do?
Prevents tissue destruction
What diseases are associated with NALP3 mutations?
- Familial cold urticaria
- Muckle-Wells
- Neonatal onset multisystem inflammatory disease
It is important to avoid which medication in hereditary angioedema?
ACE inhibitors
What does exulizumab do?
Monoclonal C5a antibody, useful for PNH and aHUS
What is a Type 1 reaction?
Immediate IgE mediated reaction e.g. anaphylxis, urticaria, angioedema, anaphylactic shock, bronchial asthma, rhinitis, conjunctivitis
What is a Type 2 hypersensitivity reaction?
Antibody mediated cytotoxic reaction e.g. immune haemolytic anaemia
What types of allergy do you do intradermal testing for?
Drug allergy
Insect venom allergy
What is a Type 3 hypersensitivity reaction?
Immune complex mediated e.g. serum sickness, vasculitis
When is tryptase highest?
Peaks 60-90mins after anaphylaxis, can be taken 15mins after, helpful up to 3 hrs but may be high for up to 5hrs
What autoantibodies are positive in polyarteritis nodosa?
None - trick question!
Associated with strep infection, Hep B, chronic hepatitis
What does AIRE actually do?
Induces expression of self-proteins in thymic medullary cells
Regulates central tolerance
How does toxic shock syndrome happen?
Bacteria produce a superantigen toxin that allows the nonspecific binding of MHC II with T-cell receptors, resulting in polyclonal T-cell activation
MHC class I pathway is for…?
- 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
What type of reaction is Steven-Johnson Syndrome?
- 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
What is the difference between SJS and TEN?
- TEN more severe
- >30% of body
Why don’t B cells develop in Bruton’s (XLA)?
Because there is no bruton tyrosine kinase i.e. no SIGNALLING (critical signalling molecule)
What would you expect from a TH2 response?
High IgE
High eosinophils
Raised IL4
Raised IgG4
Diseases with inflammasome reaction respond to blockade of….
IL-1 which is anakinra
