Respiratory - Bronchiectasis/CF/COPD

Question 1

Tree in bud may represent

Answer 1

Non tuberculous mycobacterial infection

Question 2

How does macrolide help with chronic inflammation in bronchiectasis

Answer 2

1. Impair production of proinflammatory cytokines
2. Inhibiti neutrophil adhesion to cells
3. Reduce muchs secretion from airways
4. Inhibiti quorum sensing signals Decreasing biofilm development
5. Decrease refkux
6. Impair epithelial cell growth and fibroblast migration

Issues:
1. Increased resistance
2. Increased of non tuberculous mycobacteria (impaired inteacellular killing of mycobacterua in macrophages)
3. Prolong qt interval

Question 3

Current cftr modulators

Answer 3

1. Ivacaftor - CFTR potentiator - opens channel of protein (mainly for type 3)
2. Tezacaftor - CFTR corrector / moves protein to the cell surface
3. Elexacaftor - CFTR corrector / moves protein to the cell surface
4. Ivacoftor plus tezacaftor for homozygous DF508 mutations
5. Elexacaftor/ivacaftor/tezacaftor for heterozygous DF508 mutations

Question 4

Primary ciliary dyskinesia - inheritance and clinical features

Answer 4

Autosomal recessive. Absence or shortening of the Dynein arms in cilia. Presents with bronchiectasis, recurrent otitis media, chronic sinusitis, frontal sinus agenesis.

Subset of this is kartageners syndrome

Question 5

Kartagener’s syndrome triad

Answer 5

Bronchiectasis from primary ciliary dyskinesia, situs invertus, sinusitis.