Immunodeficincies

Question 1

What are immunodeficines?

Answer 1

• caused by defects in one or more components of the immune system
• may lead to serious diseases or syndromes
• classfiied as primary or secondary
• date difficult to estimate as no current screening programme at birth

Question 2

What are primary (congential) immunodeficiencies?

Answer 2

• a condition resulting from a genetic or developmnetal defect
• defect is present from birth, and most likely to be inherited
• may not be clinically obsered until later in life

Recurrent infections

Present with severe infections with unusualy pathogens and in unsual sites

2 or more of the warning signs could indicate PID

Causes:

• they may affect either the innate or adaptive immune function
• defects in the innate immunity are generally caused by defect in phagocytic or complement function
• lymhpid cell disorders may affect T cells or B cells or both

Question 3

What are secondary immunodeficines?

Answer 3

• orginate as a result of malnutrition, cancer, drug treatment or infection
• much more common

Question 4

How can haematopoiesis be linked to PID?

Answer 4

• consequences of the defect depend upon the number and type of immune system components involved
• defects in earlier stem cells affect the entire immune system
• defects in later stage show more of a restricted pathology

Question 5

How does gene therapy work?

Answer 5

Question 6

Describe HIV-AIDS

Answer 6

• it is a secondary immunodeficiency
• infections are the major cause of death in AIDS patients
  
  • fungal, viruses, parasites and intracellular bacteria
• treatment: HAART and PrEP