Primary Immunodeficiency

Question 1

What are the components of Spefiic immunity

Answer 1

Antibodies

Cellular immunity

Question 2

What is cellular immunity

Answer 2

Cell-mediated immunity is the activation of phagocytes, antigen-specific cytotoxic T-lymphocytes, and the release of various cytokines in response to an antigen.

Question 3

What are the components of non-specific immunity

Answer 3

Complement

Phagocytes

Question 4

What is the complement system in immunity

Answer 4

The complement system, also known as complement cascade, is a part of the immune system that enhances the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inflammation, and attack the pathogen’s cell membrane.

Question 5

What does primary immunodeficiency increase you chances of

Answer 5

tumours

infection

Question 6

what is repeated infection with encapsulated bacteria a sign of

Answer 6

defective antibody production

Question 7

What antibody deficiency leads to repeated respiratory infections and what bacteria is responsible

Answer 7

igG and igA

Pneumococcus or haemopgilus influenza

Question 8

What does infection with staphylococci, gram nergative bacteria or fungi indicate a deficiency in

Answer 8

Phagocytes

Question 9

What defeciency predisposes to infection from intracellular organims such as protozoa, viruses, intracellular bacteria

Answer 9

T cells or macrophages

Question 10

T cell deficiency predisoses to infection from what

Answer 10

Mycobacteria

intracellular organisms such as protozoa, viruses, intracellular bacteria

Question 11

What is reactivated herpes virus infection linked to?

Answer 11

T cell immunodeficiency

Question 12

What does a defective Th17 pathway cause

Answer 12

Recurrent candida infection

Question 13

What are 3 general causes of primary immunodeficiency

Answer 13

Mutations
polymorhisms
polygenic disorders

Question 14

How severe is each different cause of primary immunodeficiency

Answer 14

Mutations - severe
polymorhisms - moderate
polygenic disorders - mild (autommunity)

Question 15

What condition can many mutations cause

Answer 15

Severe combined immunodeficiency

Question 16

Describe SCID

Answer 16

infants die within 2 months without treatment

stem cell transplant is cure

Question 17

What is an example of a polymorphism

Answer 17

Human Leakcyte antigen

Mannan-binding lectin

Question 18

What are common polygenic disorders

Answer 18

Common variable immnodeficiency (most common)

igA deficienty

Question 19

Describe CVID

Answer 19

low levels of igG
Varible levels of a, M and B and T
causes recurrant resp tract infections
autoimmunity is common

Question 20

Whats the clinical presentation of SCID

Answer 20

defective B and T cells and therefore develop infectinos in first few weeks of life
diarrohoea
unusual rashes
low lymphocytes count

Question 21

When does CVID present

Answer 21

later life

Question 22

What is treatment of primary immunodeficeicny

Answer 22

Mild - prohylactic antibiotics

Severe - immunoglobulin replacement therapy

Question 23

Treatment of confirmed SCID

Answer 23

Avoid live vaccines 
stem cell transplantation 
gene therapy (when stem cell not available_

Question 24

What is more common, primary or secondary immunodefieicny

Answer 24

Secondary

Question 25

When does secondary immnodeficieny occur

Answer 25

When synthesis of key immune componenents is supressed (bone marrow infiltration) or their loss is acccelerated (kidney issues)