COPD, Bronchiectasis, CF

Question 1

Emphysema

Chronic Bronchitis

Answer 1

Answer 2

Airways responsiveness

FEV1 measured yearly, decline post bronchodilator noted

Best predictor of survival

Cigarette smoking

Low body-mass index (BMI ≤21)

HIV infection

Increased airway bacterial load

Decreased exercise capacity

Peak oxygen consumption (VO2), measured by cardiopulmonary exercise testing

Elevated C-reactive protein (>3 mg/L)

Male gender

Chest computed tomography (CT) showing presence of emphysema

Question 3

General Treatment Stratergy

Answer 3

Step 1 –> Relievers

SABA - Salbutamol, Ipratropium

Step 2 –> Long acting

LAMA - Umeclidinium, Tiotropium

LABA - Indacterol

Step 3 –> Dual therapy - LAMA + LABA

Indacaterol/glucopyrronium - Ultibro

Tiotropium/Olodaterol - Spiolto

Aclidium/formoterol - Brimica Genuiar

Step 4 –> Add ICS (either triple combo OR ICS/LABA + LAMA)

ICS/LAMA: Breo Ellipta - Fluticasone/vilanterol, Symbicort - Budesonide/fomoterol, Seretide - Fluticasone/salmeterol

Triple - Trelegy - Fluticasone/Umeclidinium/Vilanterol **IMPACT Study - reduced hospitalization but increased risk of pneumonia

Question 4

Benefit of;

1. Smoking cessation
2. Pul rehab
3. O2

Answer 4

1. Only continuous >16 hours a day to get mortality benefit but min. Portable O2 no mortality benefit. Improved QoL, doesnt improve hospitalizations
2. Smokin cessation - mortality benefit
3. Pul rehab - improved exercise capacity, QoL and hospitalizations

Question 5

Indications for NIV

Answer 5

Bipap indicated in hypercapneoic acidosis (pco2 > 45 or ph <7.30) or if RR >30.

Question 6

Kartagener’s Syndrome

Answer 6

Kartagener’s syndrome (KS) is a rare autosomal recessive genetic disorder with a prevalence of 1:32,000, constituting about 50% of the primary ciliary dyskinesias (PCD) and characterized with a course including the triad of sinusitis

Question 7

CF

Genetics, Prevalence

Pathogenesis

Answer 7

Autosomal recessive, carrier 1 in 25, prevalence 1:2500

Mutation in CFTR gene –> influx of sodium and chloide in lumen

Question 9

CF - clinical manifestations and diagnosis

Answer 9

CF - chronic productive cough, malabsorbtion, FTT, hyperviscous secretions –> multiple infections, infertility

Sweat tests - high sweat chloride (>60), CFTR genotyping

Question 10

CF management

non pharmaco

pharmaco

Answer 10

Non pharmaco - Sputum clearance, Nutrition, Chest physio

Pharmaco -

Question 11

Ivacaftor

MOA

Answer 11

potentiator, ie increases the time that the CFTR chloride channel remains open. It is only effective for patients with the gene mutation G551D

Question 12

Lumacaftor

MOA

Answer 12

corrector, ie increases the amount and function of cystic fibrosis transmembrane conductance regulator (CFTR) protein on the cell surface