Immune Dis and Defic.

Question 1

In the first 2-3 years of life, what is a “healthy” # of otitis media and gastroenteritis?

Answer 1

More than 6 episodes of otitis media

or
More than 2 episodes of gastroenteritis/yr

Question 2

4 components of immune fxn

Answer 2

1. Anatomic
2. Phagocytes
3. Cellular immunity
4. Humoral immunity

*there are diff tests you can run on all of these - refer to slide near end of lecture

Question 3

Humoral immunity

Answer 3

specific ab (B cells)
- Ig_ 

Complement

Question 4

Public vs Private Immunodeficiency phenotypes

- Which one can wane with age?

Answer 4

1. You have a broad syndrome/disease that leads you suceptible to multiple infxns w/ multiple organisms
2. Private phenotype: you have a very specific mutation/selective defect that leads you suceptible to one infxn
   * Private phenotype can wane with age: can develop antibodies

Question 5

Most common source of immunodeficiency?

Answer 5

Usually secondary:

Malnutrition

• Vit A def
• Iron def
• Micronutrient def
• less caloric intake

–> increased risk for infxn and decreased outcome of infxn

Question 6

2nd and 3rd largest source of immunodef.

Answer 6

2nd: HIV-1/AIDS
- 35 mill infected

3rd: Age

Question 7

Phagocytes (WBCs) mainly consist of which two types of cells?

Answer 7

PMNs (60%)
- neutrophils

Lymphocytes (30%)
- 1500-4000

Monocytes (10%)
- macrophages

Question 8

Sources of infxn with chemotherapy-associated Neutropenia

Answer 8

1. Intestines
   - transcytosis from epithelium
   - perirectal abcess
2. Periodontal
3. Skin/Catheters

*pts may have less inflammation (tumor, rubor, calor, dolor)

Question 9

Chronic Granulomatous Disease (CGD) pts have _____ phagocyte number, but _______ fxn.
- Presentation

Answer 9

Sufficient phagocyte number, but decreased fxn
- Defect in NADPH oxidase (oxygen radicals decreased)

Can die young from infxn (fam hx)

*more likely to get uncommon organisms

Question 10

Conductor/director of immunity

Answer 10

CD4+ T cells

Tells everybody what to do and when to do it. They dont actually kill themselves.

Question 11

causes of decreased cellular immunity

Answer 11

1. Malnutrition
2. Oral steroids
3. Immunotherapy
4. Chemotherapy
5. Transplantation
6. HIV/AIDS

Question 12

Causes of pneumonia in:

Elderly

Answer 12

1. S. pneumoniae
2. H. flu
3. Influenza (Virus winter)

Question 13

Causes of pneumonia in:

Young adult

Answer 13

1. M. pneumoniae
2. C. pneumoniae
3. S. pneumoniae

Question 14

Causes of pneumonia in:

AIDS

Answer 14

S. pneumoniae

P. jirovecii (PCP)

Question 15

Causes of pneumonia in:
BMT

Nosocomial

SE Asia/Africa

Answer 15

BMT: Aspergillus sp

Nosocomial: GNR

SE Asia/Africa: TB

Question 16

Protozoans you are more likely to get with impaired cellular immunity

Answer 16

1. Toxoplasma

2. Cryptosporidium

Question 17

Helminths you are more likely to get with impaired cellular immunity

Answer 17

Strongyloides

Question 18

Viruses you are more likely to get with impaired cellular immunity

Answer 18

HSV
CMV
Varicella

Question 19

Secondary immunodeficiecy in developing countries vs developed countries

Answer 19

Developing country:

1. Malnutrition
2. HIV-1/AIDS
3. Measles
4. Age

Developed country:

1. Immunotherapy (steroids)
2. HIV-1/AIDS
3. Cancer Therapy
4. Transplantation

Question 20

3 pathogens with invasive polysacc capsules

• which antibody is going to recognize it?

Answer 20

1. strep pneumo
2. G. mening
3. H. flu

IgG2 - recognize polysaccharides

Question 21

Antigen binding region

Answer 21

Fab (Fragment Antigen Binding)

• variable region
• each is unique

Question 22

Which region of antibody defines isotype?

Answer 22

Fc Constant region

• activates compliment
• binds phagocytes via Fc receptors

Question 23

Antibody deficiency leaves you susceptible to

Answer 23

1. Respiratory infxn
2. Encapsulated bacteria
3. Viruses (enterovirus)
4. Giardia

Question 24

IgA1,2 are found where?

Answer 24

Mucosal>blood

Question 25

Hyper IgE syndrome

Answer 25

Job's syndrome

Question 26

Most common primary immunodefienciency

Answer 26

Selective IgA deficiency - Fam hx *normally you produce more IgA than IgG and IgM - no mas Lots of people have this, m: there is very little associated increased infxn rate (look for concurrent infxn: like low IgG2)

Question 27

Which complement stimulates opsonization/phagocytosis

Answer 27

C3 Complements act with antibodies to "opsonize" bacteria and facilitate lysis of GNR or killing by phagocytes

Question 28

List the primary immunodefiency in adults

Answer 28

1. IgA deficiency 2. IgG2 subclass def 3. CVID - common variable immunodeficiency 4. Hyper-IgE syndrome (job's syndrome) 5. Cystic fibrosis *most primary immunodeficiencies are present in childhood and a result of single gene defects

Question 29

Immunodeficiency is an increased incidence and severity of what 3 things?

Answer 29

1. INfection 2. Malignancy 3. Autoimmunity

Question 30

How id neutropenia defined?

Answer 30

PMNs are usually

Question 31

In the first 10 yrs, what is the healthy number of URI/yr?

Answer 31

less than 6-8 URI/ yr

Question 32

Which complement deficiency is most common? Which complement deficiencies presents with pyogenic infxns? Which with serial Neisserial infections?

Answer 32

C2 pyogenic: C1-4 (mainly C3) Neisserial: C5-9

Question 33

Which complement def presents with hereditary angioedema?

Answer 33

C1 esterase inhibitor deficiency | - swelling is similar to hives but it is under the skin.

Question 34

Which complement def. presents with complement mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria?

Answer 34

DAF (GPI- anchored enzyme) deficiency Decay accelerating factor helps prevent complement activation on self cells

Question 35

Most common serious primary immuno defect in adults

Answer 35

Common variable immunodeficiency (CVID)

Question 36

Low fxn in these tests are indicative of what type of immunodeficiency - Phagocytic, cellular, or humoral immunity? 1. NBT 2. Skin tests 3. Anti RBC 4. Vaccination

Answer 36

1. NBT: phagocytic - sufficient phagocyte # but decreased fxn 2. Skin tests: Cellular 3. Anti RBC: humoral 4. Vaccination: Humoral

Question 37

Low # in these tests are indicative of what type of immunodeficiency - Phagocytic, cellular, or humoral immunity? 1. Lymphs

Answer 37

1. Lymphs less than 1500 | - cellular immunodef.