16.3. Paediatric Infectious Diseases - Primary Immunodeficiencies

Question 1

What are Primary Immunodeficiencies?

Answer 1

A Group of >300 Rare, Chronic Disorder in which Part of the Body’s Immune System is Missing / Functions Improperly

Question 2

What causes Primary Immunodeficiencies?

Answer 2

Single Genetic Defects

Question 3

What do Primary Immunodeficiencies affect?

Answer 3

1. A Single Part of the Immune System

2. More Components of the Immune System

Question 4

What are the 4 Main Components of the Immune Defense Mechanism?

Answer 4

1. B-Cells and Antibodies - Humoral, Specific Immunity
2. T-Cells - Cellular, Specific Immunity
3. Phagocytes - Innate Immunity
4. Complement System - Innate Immunity

Question 5

What Characterises Antibody Deficiencies?

Answer 5

1. Absence of Mature B-Cells

2. Deficiency of 1 or More (Sub)Classes of Antibodies - IgG / IgA / IgM / IgG2

Question 6

What Characterises Cellular Immunodeficiencies?

Answer 6

Impaired T-Cell Function / Absence of Normal T-Cells

Question 7

What Characterises Innate Immune Disorders?

Answer 7

1. Defects in Phagocyte Function

2. Complement Deficiencies

Question 8

What are indicators that there is a Primary Immunodeficiency?

Answer 8

1. Severe
2. Persistent
3. Unusual
4. Recurrent
5. Runs in the Family

Question 9

What is Indicative of Primary Antibody Immunodeficiency?

Answer 9

Recurrent Bacterial Infection of the Upper / Lower Respiratory Tract:

1. S. Pneumoniae
2. H. Influenzae

Question 10

What is Indicative of Primary Cellular Immunodeficiency?

Answer 10

Unusual / Opportunistic Infections + Failure to Thrive:

1. Pneumocystic Jirovecii
2. CMV (Pneumonia)

Question 11

What is Indicative of Primary Innate Immune Disorders?

Answer 11

1. Defects in Phagocyte Function:
2. a) S. Aureus - Sepsis / Skin Lesions / Abscesses
3. b) Aspergillus - Lung / Bone / Brain
4. Complement Deficiencies - N. Meningitidis