Immunology

Question 1

what is Kostmann Syndrome

Answer 1

Severe chronic neutropenia

Question 2

clinical presentation of Kostmann syndrome

Answer 2

Infections, usually within 2 weeks after birth
Fever
Failure to thrive
Oral ulceration

Question 3

examples of opsonins

Answer 3

Complement C3b
IgG
CRP

Question 4

what are examples of phagocyte receptors

Answer 4

Fc

CR1

Question 5

features of Chronic granulomatous disease

Answer 5

recurrent deep bacterial infections
recurrent fungal infections
failure to thrive 
Lymphadenopathy and hepatosplenomegaly
Granuloma formation

Question 6

what is an investigation for chronic granulomatous disease

Answer 6

NBT test

Question 7

defects in what network is linked to susceptibility to intracellular bacteria

Answer 7

IL 12 - gIFN

Question 8

how can phagocyte recruitment be investigated

Answer 8

FBC
Presence of pus
Expression of neutrophil adhesion molecules

Question 9

is there pus is chronic granulomatous disease

Answer 9

yes

Question 10

what is Recricular dysgenesis

Answer 10

Defects of haemopoetic stem cells
Failure of production of;
neutrophils
lymphocytes
monocyte/macrophages
platelets

Question 11

what is the only treatment of recricular dysgenesis

Answer 11

bone marrow transplantation

Question 12

what is severe combined

immunodeficiency

Answer 12

Failure of production of lymphocytes

Question 13

what are abnormal presentations of SCID

Answer 13

unusual skin diseases

family history of early infant death

Question 14

3-4 month old baby gets infection but is fine after - what disease is this

Answer 14

transient hypogammaglobulinaemia of infancy

Question 15

what is transient hypogammaglobulinaemia of infancy

Answer 15

immune systems of 3-4 month old babies slow to mature

Question 16

what is DiGeorge syndrome

Answer 16

development defect of 3rd/4th pharyngeal pouch

Question 17

features of DiG syndrome

Answer 17

Low set ears abnormally folded ears, high forehead,
cleft palate, small mouth and jaw
Hypocalcaemia secondary to hypoparathroidism
Oesophageal atresia
T cell lymphopenia
Complex congenital heart disease

Question 18

what is the IL12-gIFN network pathway

Answer 18

Infected macrophages stimulated to produce IL12
IL12 induces T cells to secrete gIFN
Then gIFN feeds back to macrophages and neutrophils
stimulates production of TNF
activates NADPH oxidase

Question 19

what is essential investigation in T cell deficiencies

Answer 19

HIV test

Question 20

what is Bruton’s X-linked hypogammaglobulinaemia

Answer 20

failure to produce mature B cells
no circulating B cells
no plasma cells
no circulating antibody after first 6 months

Question 21

what are the 4 types of hypersensitivity reactions

Answer 21

Type I: Immediate hypersensitivity
Type II: Direct cell killing
Type III: Immune complex mediated
Type IV: Delayed type hypersensitivity

Question 22

what is angioedema

Answer 22

Self-limited, localised swelling of subcutaneous tissues or mucous membranes

Question 23

what is Spontaneous mast cell degranulation

Answer 23

Mast cells in the skin become very twitchy, if you touch them they degranulate.

Question 24

what are non-allergic causes of Spontaneous mast cell degranulation

Answer 24

drugs - opiates, aspirin, NSAIDS
thyroid disease
idiopathic
physical urticaria

Question 25

what is Samter's triad

Answer 25

asthma, nasal polyps and salicylate sensitivity

Question 26

what test is the gold standard for diagnosing allergy

Answer 26

skin-prick test

Question 27

what are anti-histamines

Answer 27

H1 receptor antagoinsts

Question 28

what is used in anaphylaxis and how does it work

Answer 28

adrenaline | Acts on B2 adrenergic receptors to constrict arterial smooth muscle

Question 29

what are anaphylotoxins

Answer 29

Fragments of complement proteins released after activation | increase permeability of blood vessels

Question 30

what are some examples of Type II hypersensitivity diseases

Answer 30

Goodpasture’s syndrome Guillan Barre syndrome Systemic lupus erythematosus (SLE)

Question 31

what is acute hypersensitivity pneumonitis

Answer 31

Immune complexes deposited in the walls of alveoli and bronchioles

Question 32

what causes the wheeze in hypersensitivity pneumonitis

Answer 32

inflammation of terminal bronchioles and alveoli caused by activated phagocytes and complement

Question 33

what causes breathlessness in hypersensitivity pneumonitis

Answer 33

alveolitis caused by activated phagocytes and complement - results in decreased efficiency of gas transfer

Question 34

what causes malaise, pyrexia in hypersensitivity pneumonitis

Answer 34

systemic manifestation of inflammatory response

Question 35

what causes renal dysfunction

Answer 35

Deposition of IgG immune complexes

Question 36

what is the mechanism of type 4 hypersensitivity

Answer 36

Initial sensitisation to antigen - generation of “primed” T cells Subsequent exposure - activation of previously primed T cells - recruitment of macrophages, other lymphocytes, neutrophils - release of proteolytic enzymes, persistent inflammation

Question 37

what is variolation

Answer 37

The same organism is being administered as the one that can cause disease, but the route of administration is different

Question 38

when is there active transport of maternal IgG

Answer 38

3rd trimester

Question 39

what does breast milk contain especially colostrum

Answer 39

IgA

Question 40

what is IgA important for

Answer 40

colonisation of infant gi tract

Question 41

what class do all nucleated cells express

Answer 41

Class I

Question 42

what cells express Class II

Answer 42

Specialised antigen-presenting cells

Question 43

acute cellular rejection mechanism

Answer 43

Recognition of donor antigens by CD4+ cells Activation of CD4+ cells Production of cytokines Recruitment and activation of macrophages and neutrophils Type IV hypersensitivity response

Question 44

what are the symptoms of acute cellular rejection

Answer 44

Kidney transplant: Rise in creatinine, fluid retention, hypertension Liver transplant: Rise in LFTs, coagulopathy Lung transplant: breathlessness, pulmonary infiltrate

Question 45

hyperacute rejection

Answer 45

Rapid destruction of graft within minutes-hours | Mediated by pre-formed antibodies that react with donor cells

Question 46

why would an individual have preformed antibodies against donor cells

Answer 46

1) blood group antigens - naturally occurring preformed antibodies, bind surface molecules found on everything 2) HLA antigens - arise through previous exposure

Question 47

when does hyper acute rejection occur

Answer 47

recipient has pre-existing anti-donor HLA antibodies | recipient has different blood group

Question 48

what is the difference between hyper acute and acute vascular rejection

Answer 48

hyper acute the antibody is already there | vascular rejection antibody is made after transplantation

Question 49

what is chronic allograft failure

Answer 49

Cellular proliferation of smooth muscle of vessel wall - leads to blood vessel getting smaller. Occlusion of vessel lumen

Question 50

what are side effects of immunosuppressive agents

Answer 50

Drug toxicity Infection Malignancy (cancer) Atherosclerosis

Question 51

what are examples of side effects of immunosuppressive agents

Answer 51

Nephrotoxicity after non-renal transplantation | Ciclosporin associated gingival hypertrophy

Question 52

Form of SCID, causes skin rashes and infection across whole body

Answer 52

Graft versus host disease