Immunodeficiency

Question 1

secondary IDs

Answer 1

malnutrition, HIV/AIDS,

Question 2

primary IDs

Answer 2

lymphicyte maturation. combined deficiencies, phagocytic defects, complement defects and immune regulation defects

Question 3

lymphocyte maturation disorders

Answer 3

agammaglobulinemia (Burtons)
SCID

Question 4

phagocytic disorders

Answer 4

chronic granulomatous disorders
leukocyte adhesion defiency

Question 5

immune regulation disorders

Answer 5

IPEX, ALPS and FHL

Question 6

complement disorders

Answer 6

class II: angioedema
Class IV: asymptomatic

Question 7

Maintenance of peripheral tolerance

Answer 7

tTreg; apoptosis; anergy; immune prvileged sites; antigen sequestration

Question 8

how do immune privilged sites maintain immunosuppresion

Answer 8

production of TGFb
lack conventinal lymphatic drainage to avoid infiltration
physical barriers
expression of FAS to induce apoptosis

Question 9

camptothecin is an inhibitor of

Answer 9

topoisomerase

Question 10

activation of psindle assembly checkpoint

Answer 10

using MT inhibitors results in defective spindle-kinetochore attachment

Question 11

why are cancer stem cells challengin to target

Answer 11

replicate slowly, reason for relapse post treatment - residual tumour cells in body

Question 12

inhibit protein synthesis

Answer 12

deaminate key AAs to inhibit protein synthesis (L-asparaginase to inhibit protein synthesis)

Question 13

inhibit dna rpelication

Answer 13

inhibit toposiomerase act (camptothecin) –> induce dsdna breaks and adducts
croslsinking agents (cisplatin) to inducer strand crossing, dsdna breaks in s phase

Question 14

inhibit dna synthesis

Answer 14

induce adduct formation (pyrmidine dimers)
inhibit purine synthesis

Question 15

inhibit division

Answer 15

target tubulin to induce apoptotis
inhibit cyclin/kinase complexes to prevent pro-mitotic signalling

Question 16

inhibit differentiation

Answer 16

retinoic acid to neuroblastoma cells

Question 17

cros slinking agents

Answer 17

cisplatin

Question 18

diagnosis

Answer 18

use MIBG = noradrenaline analogue taken up by neuroblastoma cells
labelled w radioactive iodine then injected in body + scan to detect

Question 19

bragg peak

Answer 19

protons deposit most energy at end of their range

Question 20

pros of proton ptherpay

Answer 20

efficient dna direct damage
if can target precisely –. good bc less damage to nromal cells as release of energy in 1 spot

Question 21

pros of photon beam therapy

Answer 21

use in cancer near brain ,spine, heart and other vital organs challenging to target

Question 22

surgery for cancer eradication

Answer 22

Remove primary tumours + lymph nodes + limit spread

Question 23

inhibiting kinase pathways

Answer 23

ras –> raf –> mek –> erk
erk = tf, uoregulates cyclin D allowing progresison thru cell cycle

Question 24

MEK inhibitor

Answer 24

trametinib

Question 25

DDR| characteristics

Answer 25

broad and integrated, highly specific & highly sensitive

Question 26

DDR: ssbs signal for

Answer 26

BER, PARP protein crucial

Question 27

DDR: dsdna breaks signal for

Answer 27

NHEJ or HR, BRCA crcucial

Question 28

alloimmunity

Answer 28

immune response made by host against non self anitgens (typically derived from transplanted organs)

Question 29

SCID - ex of geneic defects leaidng to scid

Answer 29

mutations affecting: - thymocyte survival antigen receptor complex receptor subunits of cytokine receptors

Question 30

IPEX describe

Answer 30

defective FOXP3 gene essential for Treg development; lack of Tregs limits elimination of selfreactive effector T cells

Question 31

treatment of digeroge anomaly

Answer 31

thymus transplant to thigh

Question 32

ALPS

Answer 32

mutation in Fas or it sligand (death receptor) inhibits apoptosis of selfreactive T cells leading to uncontrolled lymphocyte proliferation

Question 33

FHL

Answer 33

defective lytic granule release lymphocytes release IFNy instead of cytokines leading to uncontrolled macrophage proliferation and phagocytosis --> tissue damage

Question 34

chronic granulomatous disease

Answer 34

defective NADPH oxidase, failure of phagocytes to produce ROS (superoxide ions) in ohagosomes required to degrade bacteria

Question 35

leukocyte adhesion deficiency

Answer 35

mutation affecting cell surface receptors on phagocytes, limiting their ability to slow down and adhere to epithelium in vessel; no intravasation

Question 36

immunsuppressant drug general mechainsm

Answer 36

inhibit mast cell degranulation reduce dendritic cell activation reduced secretion of inflam cytokines

Question 37

chronic immunsuppressant use side effects

Answer 37

hirsutism, long wound healing, striae

Question 38

AID - main features

Answer 38

chronic self pepertuating mostly tissue specific

Question 39

classes of AID

Answer 39

organ specific - T cell mediated organ spec - antibody mediated systemic

Question 40

2 main causes of AID

Answer 40

breakdown of self tolerance allowing escape of self reactive lymphocytes genetic susceptibility (MHC locus)

Question 41

ex of genetic susceptibility gene AID

Answer 41

MHC II allele DQ8 in type 1 diabetes