Immunity

Question 1

Causes of toxic shock syndrome

Answer 1

Staph aureus

Group A strep

Question 2

Which lab test can you do in toxic shock syndrome?

Answer 2

CK - raised

Question 3

Quantitative test of B cell function particularly in children over 2

Answer 3

Antibodies to polysaccharide vaccines

Question 4

Test of T cell function assessing mitogen proliferation

Answer 4

PHA (phytohemagglutinin)

Question 5

What is PHA?

Answer 5

Stimulus to T cell proliferation

Question 6

Diagnostic test for SCID with neurological abnormalities

Answer 6

PNP enzyme activity

Question 7

What does positive hep B surface antigen indicate?

Answer 7

Either acute infection or chronic infection

Question 8

How do you prove chronic hep B infection?

Answer 8

Either two positive hep B surface antigen tests three months apart, or simultaneous demonstration of HbsAg and IgG core antibody to core antigen (anti HBc)

Question 9

Which mothers are at particular risk of passing hepatitis B on to their babies?

Answer 9

Those who have detectable ‘e’ antigen - high rates of active viral replication

Question 10

What should infants of hep B ‘e’ antigen positive mothers receive at birth?

Answer 10

Immunoglobulin (HBIG) and immunisation, in separate limbs

Question 11

What should infants of hep B surface antigen positive mothers receive at birth?

Answer 11

Vaccination within 48 hours and then complete the course

Question 12

How long should you observe a patient post anaphylaxis?

Answer 12

6-12 hours

Question 13

6 most important opportunistic infections in paediatric HIV?

Answer 13

CMV retinitis
PCP
Disseminated MAC
Oesophageal candidiasis
Cryptococcal meningitis
Chronic cryptosporidium enteritis

Question 14

Blood tests in SCID?

Answer 14

Lymphopenia
Low T cell numbers
Poor functional activity of T cell

Question 15

Suspect if delayed separation of umbilical cord and no pus?

Answer 15

Leucocyte adhesion defect

Question 16

How do you diagnose cyclical neutropenia?

Answer 16

Twice weekly FBC for 8 weeks

Question 17

How is cyclical neutropenia treated?

Answer 17

GCSF, if treatment is needed

Question 18

What is the mutation in Bruton’s?

Answer 18

btk gene (bruton tyrosine kinase) causing defective pre-B to B-cell transformation

Question 19

Presents with gingivostomatitis at regular intervals

Answer 19

Cyclical neutropenia

Question 20

Presents at 6m-2y with recurrent bacterial infections, reduced or absent lymphoid tissue, IgG, M, and A all low and very low or absent CD19/CD20

Answer 20

Bruton’s X linked agammaglobulinaemia

Question 21

What is the treatment for Bruton’s

Answer 21

Regular IVIG or SCIG infusions

Question 22

Presentation with eczema, recurrent bacterial infections and purpura

Answer 22

Wiskott-Aldrich

Question 23

How is Wiskott-Aldrich diagnosed?

Answer 23

WASP expression

Question 24

Which investigations should children with Wiskott-Aldrich receive?

Answer 24

Liver-spleen scan and serum immunoglobulins

Question 25

What are the risks of Wiskott-Aldrich?

Answer 25

Serious herpes infection, malignancy, autoimmunity

Question 26

What is the treatment for Wiskott-Aldrich?

Answer 26

Platelet transfusion if indicated, BMT (can be curative)

Question 27

Presents with recurrent infections and low IgG +/- low IgA in infants

Answer 27

Transient hypogammaglobulinaemia of infancy

Question 28

Immune-related blood tests in HIV

Answer 28

Raised IgG | Reversed CD4-CD8 ratio

Question 29

Presents early in infancy with failure to thrive, chronic diarrhoea, recurrent infections, infection with unusual organisms and chronic candidiasis

Answer 29

SCID

Question 30

What investigations should be done in suspected SCID?

Answer 30

Serum immunoglobulins Lymphocyte subsets PHA stimulation tests

Question 31

Cause of chronic granulomatous disease

Answer 31

Inherited defect in the NADPH dependent oxidase enzyme system, unable to deal effectively with certain phagocytksed organisms

Question 32

Presents with suppurative infection, macrophage activation and formation of granulomata

Answer 32

Chronic granulomatous disease

Question 33

Test for CGD

Answer 33

Flow cytometry to assess neutrophil respiratory burst activity

Question 34

Lab tests in ataxia telangiectasia

Answer 34

Low IgA Elevated serum alpha fetoprotein Increased white cell sensitivity to irradiation T cells may be low

Question 35

Presents with very high IgE, eczema, staphylococcal abscesses

Answer 35

Hyper IgE syndrome (Job syndrome)

Question 36

Cause of roseola infantum

Answer 36

HHV6

Question 37

Cause of molluscs contangiosum

Answer 37

Poxvirus

Question 38

What should be regularly checked in children on immunoglobulin therapy?

Answer 38

LFTs Hep C status Trough Ig levels

Question 39

Test for LAD?

Answer 39

Flow cytometry for CD11b