L13 - 16

Question 1

Give examples of inherited and acquired defects compromising pulmonary defenses

Answer 1

In the innate immune system. (innate/ aquired)
Mucicilliary escalator (CF/ smoking)
Innate cellular defence (Chronic granulomatous disease/ smoking)
In the adaptive immune system. (innate/ aquired)
B Cells (CVID/ chronic lymphocytic leukaemia)
T cells (HIV/ immunosupression)

Question 2

Discuss specific treatments which may improve such compromised defenses

Answer 2

CF (concentrated extracellular mucous)
G551D - Ivacaftor (potentiator)
Tezacaftor and Lumacaftor are correctors which help folding of CFTR and hence trafficking to plasma membrane.
deltaF508 - Combination of Tezacaftor and Ivacaftor improved health.

HIV - you know!!!!

Question 3

Describe how preventative (prophylactic) strategies may be used to reduce infections

Answer 3

Antibiotic prophylaxis in myelotoxic chemotherapy when neutropenia (low neutrophil count) can be predicted both in time and severity
Antibiotic and antifungal prophylaxis for chronically neutropenic patients eg bone marrow transplant, or those with inherited defects 
Pneumocystis pneumonia (PCP) prophylaxis in HIV and immunosuppression (T helper cells compromised)