Immunodeficiencies

Question 1

Chediak-Higashi Syndrome, 4F’s

Answer 1

• Dark Skin
• Low NK, Low CTL
• Defect in forming granules
• Giant Lysosomes are created

Question 2

Three functions of immune system

Answer 2

1. Defense
2. Homeostasis
3. Surveilllance

Question 3

Consequences of leukocyte adhesion deficiencies?

Answer 3

1. Leukos can’t leave and create lesions
   Seen in gums or umbilical cord (type 1)
   Seen with selectins getting ruined (type 2)

Question 4

Presentation of late defects in complement

Answer 4

• Neisserial attacks

- Gram-Negative infections that need to be MAC’D

Question 5

Hyper homeostatic function leads to:

Answer 5

Autoimmune disease

Question 6

Presentation of early defects in complement: (C1q, C4, C2)

Answer 6

• Staph infections
• Lupus
• Scleroderma

Question 7

Dihydrorhodamine Test

Answer 7

Measure ability of white cells to be stimulated

Question 8

DiGeorge Syndrome

Answer 8

• Caused by decrease in T-cells
• Hypocalcemia
• Viruses and Fungi are big risks

Question 9

Hypo defense leads to:

Answer 9

Immunodeficiency; 65% due to antibody

Question 10

Gel-Coombs 4 Types

Answer 10

1. IgE
2. Cytotoxic
3. Ag/Ab and Complement
4. Delayed Hypersensitivity

Question 11

Secondary Immunodeficiency

Answer 11

External defects like Diabetes, AIDS, cystic fibrosis, Age, Trauma, Cancer, Drugs

Question 12

C1 inhibitor deficiency presentation:

Answer 12

-Hereditary Angioedema

Question 13

Primary immunodeficiency

Answer 13

Defects in innate/adaptive system

Question 14

Hyper defense leads to:

Answer 14

Allergy

Question 15

Three “T’s” for Molecular Biology

Answer 15

1. Transduction
2. Transcription
3. Translation

Question 16

Four Key Defects in immunodeficiency (Chronological)

Answer 16

1. Granulomatous Disease (hematopo defect)
2. SCID (Lym progen defect)
3. DiGeorge (T-defect)
4. XLA (B-defect)`