Cystic Fibrosis, Bronchiectasis

Question 1

What is Cystic Fibrosis (CF)?

Answer 1

An inherited autosomal recessive disease caused by a single gene defect on chromosome 7

Question 2

What does the CFTR gene encode?

Answer 2

The cystic fibrosis membrane conductance regulator, a transmembrane protein involved in ion transport

Question 3

What is a common diagnostic test for Cystic Fibrosis performed on newborns?

Answer 3

Heel prick test

Question 4

What does the sweat test measure in suspected CF patients?

Answer 4

Chloride concentration in sweat

Question 5

What are some common symptoms of Cystic Fibrosis in the lungs?

Answer 5

• Persistent cough
• Wheezing
• Shortness of breath
• Recurring lung infections

Question 6

What causes pancreatic insufficiency in CF patients?

Answer 6

Abnormal ion transport leading to dehydration of pancreatic secretions

Question 7

What is a significant consequence of the CFTR protein defect?

Answer 7

Impaired transport of chloride ions

Question 8

True or False: There is a cure for Cystic Fibrosis.

Answer 8

False

Question 9

What is the typical life expectancy for individuals with Cystic Fibrosis?

Answer 9

About half will live past the age of 40

Question 10

What are some gastrointestinal symptoms of Cystic Fibrosis?

Answer 10

• Large smelly stools
• Malnutrition
• Poor weight gain

Question 11

What is Meconium Ileus?

Answer 11

A serious bowel obstruction occurring in about 10% of newborns with CF

Question 12

What types of treatments are available for Cystic Fibrosis?

Answer 12

• Bronchodilators
• Antibiotics
• Mucolytics
• Digestive enzymes

Question 13

What is the role of the CFTR protein?

Answer 13

Controls the flow of water and chloride ions between membranes

Question 14

What are some common additional symptoms in CF patients?

Answer 14

• Diabetes
• Sinusitis
• Infertility

Question 15

What is the impact of CF on the liver?

Answer 15

Cirrhosis due to abnormal ion transport

Question 16

What physiological changes can occur in the musculoskeletal system of CF patients?

Answer 16

• Inspiratory muscle atrophy
• Weakness in anti-gravity muscles
• Kyphosis of the spine

Question 17

What is the significance of carrier testing for Cystic Fibrosis?

Answer 17

Determines if someone is a carrier of the faulty gene

Question 18

Fill in the blank: CF patients often experience _______ due to the absence of vas deferens.

Answer 18

Infertility

Question 19

What is the relationship between CF and sodium absorption?

Answer 19

High sodium sweat due to chloride ions not being absorbed

Question 20

What is the typical cause of death in CF patients?

Answer 20

Lung complications

Question 21

What management strategies are important for CF?

Answer 21

• Physiotherapy
• Medication
• Dietary advice
• Exercise

Question 22

What is a common psychological impact of living with Cystic Fibrosis?

Answer 22

Psychological support is often needed

Question 23

What is pneumonia?

Answer 23

An inflammatory condition of the lung leading to abnormal alveolar filling with consolidation and exudation.

Question 24

What are the four stages of pneumonia?

Answer 24

• Congestion
• Red Hepatization
• Gray Hepatization
• Resolution

Question 25

What characterizes the Congestion stage of pneumonia?

Answer 25

Vascular engorgement, intra-alveolar fluid, and numerous bacteria.

Question 26

What is the time frame for the Red Hepatization stage of pneumonia?

Answer 26

2-3 days.

Question 27

What happens during the Gray Hepatization stage of pneumonia?

Answer 27

Progressive disintegration of red blood cells with persistent fibrin exudate.

Question 28

What are common causes of pneumonia?

Answer 28

* Bacteria (e.g. Streptococcus pneumonia) * Fungi * Virus (e.g. respiratory syncytial) * Parasites * Chemical * Aspiration * Inhalation (smoke, burns)

Question 29

What are the classifications of pneumonia based on setting?

Answer 29

* Community Acquired Pneumonia (CAP) * Health Care Associated Pneumonia (HCAP) * Hospital Acquired Pneumonia (HAP) * Ventilator Associated Pneumonia (VAP)

Question 30

What is a common bacterial cause of Community Acquired Pneumonia?

Answer 30

Streptococcus pneumonia.

Question 31

What are common symptoms of pneumonia?

Answer 31

* Fever * Malaise * Muscle ache/fatigue * Coughing (productive and non-productive) * Dyspnoea * Pleuritic or chest pain

Question 32

What are less common symptoms of pneumonia?

Answer 32

* Coughing up of blood * Fatigue * Nausea/Vomiting * Diarrhoea * Wheezing * Confusion

Question 33

What are some complications of pneumonia?

Answer 33

* Lung abscess * Pleural effusions * Empyema * Septic Shock

Question 34

What vital signs indicate a medical diagnosis of pneumonia?

Answer 34

* Temperature > 37.8°C * Heart rate > 100 bpm

Question 35

What diagnostic tests are used for pneumonia?

Answer 35

* CXR * CT Scan * Blood test * Sputum culture * Pleural fluid culture * Bronchoscopy

Question 36

What is included in the general management of pneumonia?

Answer 36

* Antibiotics/Anti-Fungal medication * Oxygen support * Hydration – IV fluids * Rest * Analgesics * Cough suppressant medication * Fever-reducing medication

Question 37

What is the role of physiotherapy in pneumonia management?

Answer 37

To treat clinical signs and symptoms, assist with sputum clearance, and manage oxygen consumption.

Question 38

Fill in the blank: Pneumonia can be classified as _______ based on the setting.

Answer 38

Community Acquired Pneumonia (CAP), Health Care Associated Pneumonia (HCAP), Hospital Acquired Pneumonia (HAP), Ventilator Associated Pneumonia (VAP)

Question 39

What is bronchiectasis?

Answer 39

Permanent abnormal dilation in one or more of the lungs' bronchi, leading to mucus accumulation and increased infection risk. ## Footnote Symptoms can resemble COPD but do not always involve airflow obstruction.

Question 40

What are the key steps in the pathogenesis of bronchiectasis?

Answer 40

* Impaired mucociliary clearance * Accumulation of secretions * Bacterial infection * Increased mucus production * Impaired ciliary performance * Inflammatory response * Tissue damage * Dilated bronchi ## Footnote This process creates a vicious cycle of worsening symptoms.

Question 41

What are the causes of bronchiectasis?

Answer 41

* Idiopathic * Infection * Cystic fibrosis * Immunodeficiency * Ciliary dysfunction * Allergic bronchopulmonary aspergillosis * Inflammatory conditions * Aspiration/obstruction ## Footnote Each cause can contribute to the development of bronchiectasis.

Question 42

What are common clinical features of bronchiectasis?

Answer 42

* Cough and chronic sputum production * Dyspnoea and wheeze (75%) * Chest pain (50%) * Signs of chronic sinusitis and nasal polyps (1/3) * Recurrent exacerbations * Haemoptysis (approx. 50%) ## Footnote Haemoptysis is rarely life-threatening.

Question 43

What diagnostic tools are used for bronchiectasis?

Answer 43

* Chest x-ray * High-resolution computed tomography * Blood and sputum microbiology * Pulmonary function tests ## Footnote These tools help confirm the diagnosis and assess lung function.

Question 44

What are the three types of bronchiectasis?

Answer 44

* Saccular * Cylindrical * Varicose ## Footnote Types may be localized or widespread.

Question 45

What are symptoms of an acute exacerbation of bronchiectasis?

Answer 45

* Change in sputum production * Increased dyspnoea * Increased cough * Temperature >38.0˚C * Increased wheezing * Malaise, fatigue, lethargy * Reduced pulmonary function * X-ray changes * Changes in chest sounds ## Footnote Four of these symptoms can define an acute exacerbation.

Question 46

What treatments and management strategies are available for bronchiectasis?

Answer 46

* Physiotherapy * IV/oral/nebulised antibiotics * Bronchodilators * Steroids * Nasal sprays * Vaccinations (flu and pneumococcal) * Surgery ## Footnote These interventions help manage the condition and improve patient outcomes.

Question 47

What does the prognosis for bronchiectasis depend on?

Answer 47

Prognosis is related to lung function and the presence of infection. ## Footnote Studies indicate that prognosis for hospital-treated patients is better than for those with COPD, but poorer than for asthma patients.

Question 48

How should bronchiectasis be managed long-term?

Answer 48

Requires long-term follow-up, ideally by a respiratory specialist within a multidisciplinary team, although GPs can manage most follow-ups. ## Footnote Understanding and treatment effectiveness are crucial for patient care.