Bronchiectasis/cystic fibrosis/lung abscess

Question 1

what is the definition of bronchiectasis

Answer 1

irreversible dilation of one or more bronchi in a focal or diffuse manner due to inflammation

Question 2

what is the classification of bronchiactasis

Answer 2

cylindrical or tubular
varicose
cystic

Question 3

what is the etiology of the two types of bronchiactasis?

Answer 3

focal -> obstruction
diffuse -> infections, bacterial

Question 4

what are other causes of bronchictasis?

Answer 4

immuno def
Kartageners syndrome

Question 5

explain shortly the pathogenesis of bronchictasis

Answer 5

decreased mucociliary clearance casues secretion retention -> increased risk of infection -> infllamtion causing weakening wall -> bronchictasis -> deficitary secretion clearance ->

Question 6

what are the clinical manifestations ?

Answer 6

persistent orductive cough
with production of thick, tenacious sputum
acute exacerbations
- changes in the nature of sputum production
- increased volume and purulence

Question 7

explain the physical examination findings

Answer 7

crackles
wheezing
clubbing of the digits

Question 8

explain the diagnosis of bronchiactasis

specific findings of bronchiectasis

Answer 8

persistent chronic cough and sputum production
radiography - tram tracks
CT - tree bud
wall thickening
cysts from the wall
signet ring sign

Question 9

what is the bronchoscopy in bronchicatsis used for?

Answer 9

to asses forcal bronchiectasis

Question 10

what is pulmonary fucntional testing for in bronchiectasis?

Answer 10

obstruction dysfunction - early
obstructive/restrctive - later

Question 11

what are the treatment for bronchictasis ?

Answer 11

antibiotics
bronchial hygiene
anti inflammatory
surgery
transplnatation

Question 12

what are the complications of bronchictasis ?

Answer 12

hemoptysis due to bronchial artery embolism
respiratory failure

Question 13

what is the definition of cystic fibrosis

Answer 13

autosomal recessive transmitted disease affecting the secretion of exocrine glands
resulting in production of an inspissated secretion

Question 14

explain the clinical manifestations of CF

Answer 14

bronchiectasis with collateral tissue injury
pancreatic insufficiency
intestinal obstruction

Question 15

What is CFTR and what does it do?

Cystic Fibrosis Transmembrane Conductance Regulator

Answer 15

It’s a protein that acts as an anion channel in the cell membranes of epithelial cells (cells lining passageways in the body).

It regulates the movement of salt and water, which is essential for the consistency of secretions like mucus.

The gene for CFTR is located on chromosome 7.

Question 16

How does a faulty CFTR protein lead to the thick mucus seen in cystic fibrosis?

Answer 16

Impaired Ion Transport: In CF, the CFTR protein doesn’t work properly, so chloride ions can’t move correctly across cell membranes. This disrupts the balance of salt and water in secretions.

Dehydrated Mucus: The mucus layer becomes dehydrated and thick because water is drawn towards the higher salt concentration outside the cells.

Reduced Mucociliary Clearance: The thick mucus can’t be easily cleared by the cilia (tiny hairs that sweep mucus out of the airways), leading to blockages and infections.

Question 17

What are the effects of thick mucus accumulation in the airways of someone with CF?

Answer 17

Airway Obstruction: Thick mucus plugs the airways, making it difficult to breathe.

Chronic Infection: Bacteria thrive in the stagnant mucus, leading to repeated lung infections.

Inflammation and Damage: Ongoing infection and inflammation damage the airways, eventually causing bronchiectasis (permanent widening).

Submucosal Gland Hypertrophy: The glands that produce mucus become enlarged and filled with thick secretions.

Epithelial Damage: The airway lining is damaged and buried under layers of mucus and debris.

Question 18

what are the clinical manifestions of CF?

Answer 18

sputum secretion, like bronchiectasis, clubbing
dyspnea

Question 19

what are the rare respiratory manifestations of CF?

Answer 19

spontaneous pneumothorax
asthma
ABPA

Question 20

what are the GI manifesations?

Answer 20

pancreatic insufficiency
if not treated it causes steatorrhea

biliary duct obstruction -> biliary cirrhosis ->
- hepatic failure
- splenomegaly
- hemmorhage from esophageal carices

Question 21

what are the distal intestional obstruction syndrome in CF?

Answer 21

ingestion of large quantity of fats
in the absence of enzyme substitution therapy

Question 22

what is the diagnosis of CF?

Answer 22

lab tests -> confirmation
- DNA analysis - most valuable
- sweat analysis - shows elevated salt level
- electric potential diff - hgiher in CF
- screening - DNA test, increased trypsin

Question 23

what is the treatment of CF?

Answer 23

deit
postural drainage

Question 24

what is the definition of lung abscess ?

Answer 24

necrosis and cavitation of the lung following a microbial infection
can be single or multiple

Question 25

classify lung abscess

Answer 25

primary
- occur without an underlying condition
- most of them arise from aspiration
secondary
- occur in the setting of an underlying condition
- system or post obstructive process

Question 26

what are the risk factors of lung abcess ?

Answer 26

respiratory tract infections
diminishing of normal defense mechanisms
mechnical obstructions

Question 27

How does a primary lung abscess form?

Answer 27

Aspiration: Foreign material enters the lung.

Obstruction: The material blocks an airway, causing atelectasis (collapse of part of the lung).

Infection: Bacteria in the aspirated material multiply, leading to pneumonitis (lung inflammation).

Necrosis and Cavitation: Over 7-14 days, the infection causes tissue death (necrosis) and a cavity forms (abscess).

Question 28

Important things to remember about lung abscesses?

Answer 28

Right Lung Predominance: The right main bronchus is less angled, so aspirated material is more likely to end up in the right lung.

Delayed Radiological Signs: It takes 7-14 days after aspiration for the abscess to show up on an X-ray.

Aspiration Positions and Location:
- Supine (lying flat): Abscesses often form in the superior lower lobes or posterior upper lobes.

• Elevated/Semi-Elevated: Abscesses often form in the basal lower lobes.

Question 29

What’s the difference between a primary and secondary lung abscess?

Answer 29

Primary: Starts with aspiration (inhaling something into the lungs), usually from the mouth. The aspirated material directly causes the infection and abscess.

Secondary: Develops due to an underlying condition or complication, such as:
Bronchial obstruction (tumor, foreign body)
Immunosuppression
Cystic fibrosis
Septic emboli (infectious clots)

Question 30

What are the early symptoms of a lung abscess (1-3 days after aspiration)?

Answer 30

Sudden onset
High fever
Shivering (chills)
Altered general condition (feeling unwell)
Productive cough (coughing up phlegm)
Pleuritic chest pain (sharp pain when breathing)

Question 31

What are the symptoms of a chronic lung abscess, and how do they differ depending on the cause of infection?

Answer 31

Chronic Symptoms:
Anemia (low red blood cell count)
Weight loss

Specific Findings:
Gram-negative bacteria, Staph, amoebas: Fulminant onset (sudden and severe), purulent sputum (pus-filled phlegm), no foul smell.
Anaerobic bacteria: Develops after ~10 days, large amount of pus-filled sputum with a foul smell, sometimes bloody.

Question 32

What is pleuritic chest pain and what happens if a lung abscess ruptures?

Answer 32

Pleuritic Chest Pain:
Sudden, intense, sharp, stabbing or burning pain when breathing.
Worsens with deep breathing, coughing, sneezing, laughing.
May be referred to the neck or shoulder if inflammation is near the diaphragm.

Rupture into Pleural Cavity:
Intense chest pain, worse with deep breaths
Signs of empyema (pus in the pleural space) or pyopneumothorax (pus and air in the pleural space)
Symptoms of empyema include: dry cough, fever, chills, night sweats, malaise, weight loss, chest pain.

Question 33

what is the physical examination in lung absces?

Answer 33

• In the initial phase - signs of pneumonia
  ○ In the initial phase - signs of pneumonia
  § Dullness
  § Crackles
  
  • After the evacuation of the abscess
    ○ Cavernous breath sound on lung auscultation
    ○ Pleural fluid
    ○ If bronchial drainage is not efficient –> clubbing (after several weeks)
  • Predisposing factors
    ○ Poor dentition
    ○ Gingival disease
    Absence of gag reflex

Question 34

how is lung abscess diagnosed?

Answer 34

imaging

Question 35

what is the treatment ?

Answer 35

antibiotics