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Flashcards in Diseases Deck (21):
1

Multiple sclerosis mechanism

- immune attack attack against myelin basic sheeth that insulates neurons

2

Cause of hereditary angioedema

- C1q inhibitor deficiency

3

Deficiency of complement associated wtih system lupus erythematosis?

C1q deficiency: normal immune complexes not cleared so get deposited in tissue

4

Defieciency of Factor H/I?

- local complement activation in exposed basement membrane
- in eye and kidney
macular degeeration

5

HIV subversion of complement? [1 way]

allows C3b deposition on surface to rpomote opsoniziation and allow it to enter cell and take over

6

EBV subversion of complement? [1 way]

has glycoprotein coat similar to C3b that binds CR2 on b cells to allow entery
thats why it primarily infects B cells

7

Strep subversion of complement? [2 ways]

- has M protein that binds factor H and blocsk complement activation
- has peptidase that breaks C5a

8

Effect of deficiency in membrane attack complex

- greater susceptibility to neisseria, meningitis, gonorrhea

9

leukocyte adhesion deficiency syndrome type 1 [mech, effect]

- defect in CD18 that is part of lymphocyte function associated antigen 1 [LFA 1] and complement receptors
- get neutrophilia [high neutrophils in blood]
- lack of pus
- recurrent bacterial infection
- delayed separation of umbilical cord

10

Chronic granulomatous disease [mech, effect]

- neutrophils lack NADPH oxidase so don't have the microbicidal activity
- get opportunistic infections [staph, e coli, aspergillus]

11

Effect of RAG deficiency?

- Don't get rearrangement of B and T cell receptors
- No B or T cells
- resembles SCID
- can get spot tumors

12

Disease of deficiency in FoxP3?

- X-linked immunodysregulation [XLA]

13

Goodpastures

- collagen IV in glomerulus

14

Myestenia Gravis

- acetylcholine recetpro

15

Grave's

- hyperthyroidism
- blocks and activates TSH

16

Celiac

- not food allergy
- triggered by gluten in diet
- activated T cells make IFN-gamma, acivated gluten-specific t cells flatten epithelium

17

SCID [3 causes]

lack of T and/or B and/or NK
- X linked common gamma chain deficiency
- adenosine deaminase deficiency
- RAG1/2 defiency

18

Hyper IgM

- deficiency CD40L on T cell
- no IgG/IgA, some IgM, normal T and B numbers

19

XLA

- deficit in butron's tyrosine kinase
- prevents normal B cell development and receptor rearrangement
- smallabsent tonsils [no germinal centers]
- low/undetectable IgG/IgA/IgM
- normal lymphocyte numbers, few B cells

20

MHC Class II deficiency

- antigen presentation impaired
- no gamma-globulin
- low T cell numbers

21

stat 3 deficiency

Jobs syndrome
hyper IgE