Immunology and Infection Flashcards

1
Q

What are helper T cells also known as?

A

CD4+ T cells

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2
Q

What are cytotoxic T cells also known as?

A

CD8+ T cells

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3
Q

What is the function of helper T cells?

A

Stimulation of the B-cells to plasma cells which produce antibodies

This process takes place in the lymph-nodes

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4
Q

What os the function of cytotoxic T-cells?

A

Destruction of viruses and fungi - through destruction of the intra-cellular organisms

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5
Q

What are the features of severe combined immunodeficiency disorder?

A

Tends to present before 3 months of age

Severe lymphopenia from birth
Severe decrease or absent immunoglobulins
No antibody response to vaccinations

No B cell function or T cell function - hence small thymus and no tonsils/ adenoids noted

Recurrent infections +/- pneumonia

Tx w. bone marrow transplant

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6
Q

What is the pathophysiology of x-linked agammaglobulinaemia?

A

Defect in B-lymphocyte development which is caused by a mutation in Bruton tyrosine kinase (BTK)

BTK is involved in signalling naive B-cells to become plasma cells which then become antibodies - hence a mutation in this means that B-cells remain naive and don’t become antibodies

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7
Q

What are the features of x-linked agammaglobulinaemia?

A

Only affects boys - presents after 6 months of life (after maternal antibodies have disappeared)

Tends to present with recurrent infections

Diagnosed by flow cytometry - shows no mature B-cells
No antibodies present (IgA, IgG, IgM, IgE)

Tx - IVIg (or subcutaneous Ig)

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8
Q

What is the pathophysiology of common variable immunoglobulin deficiency?

A

Lack of B-lymphocytes or plasma cells

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9
Q

What are the features of CVID?

A

Appears during adolescence - very rarely appears before 6 years

Low IgG and IgA - variable but no absent

Normal size of large tonsils - recurrent bacterial infections
Autoimmune complications - vitiligo, RA, AIHA

Tx - IVIg (or subcutaneous Ig)

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10
Q

What are the features of transient hypogammaglobulinaemia of infancy?

A

Presents between 6-12 months of age

Commonly associated with asthma and allergies

Normal response to vaccinations

Low IgG with or without low IgA and IgM
T cell immunity is intact

Tx - supportive
IVIg in severe cases

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11
Q

What are the features of selective IgA deficiency?

A

Defined as undetectable IgA less than 5

Most common immunodeficiency
Recurrent pneumonia is present - especially with encapsulated bacteria (strep, haemophilis, m. cataralis)

Dx - low or absent IgA

Tx - abx PRN

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12
Q

What is the structure of diphtheria?

A

Gram positive bacillus

aka Klebs-Loffler bacillus

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13
Q

What is the structure of chlamydia?

A

Gram negative bacterium

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14
Q

What is the treatment of chlamydia?

A

Doxycyline PO

Second line - erythromycin/ ofloxacin

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15
Q

What is the shape of strep. pneumonia?

A

Gram positive diplococci

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16
Q

What is the shape of h. influenzae?

A

Gran negative coccibacillus

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17
Q

Which antibiotic is given to pregnant women as bacterial meningitis prophylaxis?

A

Ciprofloxacin

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18
Q

What shape is group B strep?

A

Gram positive cocci

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19
Q

What shape is E.coli?

A

Gram negative, anaerobic, rod shaped bacteria

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20
Q

What shape is listeria?

A

Gram positive

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21
Q

What are the most common causes of bacterial meningitis in the first 4 weeks of life?

A

Group B strep (gram +ve cocci)
E. coli (gram negative bacilli)
Listeria (gram positive bacilli)

22
Q

Which immunoglobulin is most associated with periodic fever syndrome?

A

IgD

23
Q

How is chronic granulomatous disease diagnosed?

A

Dihydrorhodamine neutrophil burst assay (DHR)

24
Q

What are the features of chronic granulomatous disease?

A

Frequent staph. aureus infections - most notably frequent skin abscesses

25
Q

When should mast cell tryptase levels be tested?

A

Peak is at 2 hours

Levels rise after 30 minutes so should be tested at approx. 1-2 hours post reaction

26
Q

What is the pathophysiology of XL Hyper-IgM syndrome?

A

Normally CD40 ligand is required to differentiate IgM into IgA, IgE and IgG - however in hyper-IgM syndrome there is a deficiency of CD40 therefore there is a build up of IgM and lack of other immunoglobulins

27
Q

What are the features of XL hyper-IgM syndrome?

A

Frequent infections - specifically pneumonia or PJP

Diagnosis - High or normal IgM
Low IgA, IgG, IgE
Deficient CD40 ligand on activated CD4+ T-cell lymphocytes

Mx - Ig replacement

28
Q

What are the features of hyper-IgE syndrome (aka Jobs syndrome)?

A

Recurrent infections
Coarse facial features
Eczema
Pneumatocele

Dx - elevated IgE (100 times higher than normal)

29
Q

What are the features of Wiskott-Aldrich syndrome?

A

X-lined recessive therefore only affects boys

Mutation of WASp

Triad of eczema, recurrent infections (due to absent T-cells) and thrombocytopenia

Increased risk of lymphoma (EBV related) and leukaemia

30
Q

What is the pathophysiology of leukocyte adhesion deficiency?

A

The leukocyte cannot migrate to the site of the infection due to a lack of integrin CD18 subunit which would normally adhere to the leukocyte molecule and the cellular adhesion molecules passing it to the site of infection

Infection without pus

31
Q

What are the clinical features of leukocyte adhesion defect?

A

Delayed umbilical cord seperation >2 months

No pus present at the site of the wound
Poor wound healing

Persistent leucocytosis - raised leucocyte count

32
Q

What is the investigation of choice for leukocyte adhesion defects?

A

Flow cytometric measurements of surface glycoproteins (CD18 and CD11)

33
Q

What is the pathophysiology of Chediak-Higashi syndrome?

A

It is a protein trafficking defect which means there is a deficiency of the protein involved in moving phagocytes to the lysosomes meaning impaired phase-lysosomal function

34
Q

What are the features of Chediak-Higashi syndrome?

A

Partial oculocutaneous albinism
Photophobia
Bleeding diathesis (due to low platelets)
Peripheral neuropathy
Recurrent infections

35
Q

What is the pathophysiology of chronic granulomatous disease?

A

NADPH oxidase deficiency - meaning that phagocytic cells are unable to generate hydrogen peroxide or hydroxyl radicals meaning that PMNs are unable to kill the bacteria

36
Q

What are the features of chronic granulomatous disease?

A

Pyogenic infections - abscess formation in different sites
Failure to thrive
Formation of granulomas

37
Q

How is chronic granulomatous disease diagnosed?

A

Neutrophil oxidative burst test - using rhodamine die

38
Q

What is the pathophysiology of hereditary angioedema?

A

Type 1 HAE occurs due to a deficiency of C1 esterase inhibitor

Type 2 HAE occurs due to defective C1 esterase inhibitor

39
Q

What are the most common causes of infective endocarditis in diabetic and immunocompromised patients?

A

Haemophilus
Actinobacilus
Cardiobacterium
Eikenella
Kingella

40
Q

What are the most common causes of infective endocarditis?

A

In order from most common:
Staphylococcus Aureus
Coagulase negative Staphylococcus (more likely in patients with prosthetic material) - e.g. staph. epidermidis
Streptococcus viridans

41
Q

Describe the differing hypersensitivity reactions?

A

Acute - type 1
Cytotoxic - type 2 (hours to days) - type 2
Immune mediated (weeks) - type 3
Delayed - type 4

42
Q

What is the management of HIV in children who are positive?

A

Antiretroviral therapy should be started in any child with a diagnosis of HIV regardless of their viral load and CD4 count

43
Q

What is the management of HIV in neonates born to HIV positive mothers?

A

Anti-retroviral Tx within 72 hours of birth (ideally within 12 hours)

Low risk - should start zidovudine for 4 weeks
High risk - should start combination ART (zidovudine, lamivudine, nevirapine)

44
Q

What is the treatment of choice malaria in the paediatric population?

A

Malarone (Proguanil with atovaquone)

45
Q

What is the transmission of hepatitis?

A

Hep A - contaminated water, i.e. faecal oral transmission
Hep B - vertical/ blood contact/ body fluids
Hep C - Blood
Hep D - vertical/ blood/ body fluids - requires patient to already be infected with Hep B.
Hep E - contaminated water, i.e. faecal oral transmission

46
Q

What are the characteristic findings of typhoid?

A

Relative bradycardia and fever

47
Q

Discuss complement activation by immunoglobulins?

A

IgA - alternative
IgG + IgM - classical
IgE + IgD - cannot activate complement

48
Q

What is the treatment of chlamydia?

A

Macrolides - e.g erythromycin

49
Q

Which strains of HPV are most commonly associated with neoplasm?

A

HPV 16 and 18

50
Q

What is the treatment of choice for syphilis?

A

Benzylpenicillin

51
Q

What is the treatment of choice for Gonorrhoea?

A

Ceftriaxone

52
Q
A