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Y2 - Resp > Bronchiectasis And Cystic Fibrosis > Flashcards

Bronchiectasis And Cystic Fibrosis Flashcards

1
Q

What is Bronchiectasis?

A

The chronic irreversible dilation of one or more bronchi

These deformed bronchi exhibit poor mucous clearance so theres a risk of recurrent or chronic bacterial infection

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2
Q

What is the pathological mechanism of Bronchiectasis?

A

Chronic inflammation of the bronchi cause destruction of the elastic and muscular components of the bronchial wall and peribronchal fibrosis

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3
Q

What used to be the main cause of Bronchiectasis and still is in some parts of the world?

A

Pertussis (whooping cough)

TB infection

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4
Q

What is the gold standard imaging technique used to detect Bronchiectasis?

How does it appear?

A

High resolution CT

Bronchial dilation is bigger than the adjacent blood vessel due to bronchial wall thickening (fibrosis)

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5
Q

How can Bronchiectasis appear on a CXR?

A

Tram track sign (dilated bronchi with thicken walls

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6
Q

Look at slide 6 to see the tram track sign

A
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7
Q

How does Bronchiectasis lungs appear on a CT scan?

What is the special sign that can be seen?

A

Thick white scared bronchiole walls

Signet Ring Sign

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8
Q

What is the Signet Ring sign?

A

Seen in Bronchiectasis

Where the bronchus is way more dilated than a pulmonary artery

White outer ring with central black artery

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9
Q

Go to slide 8 and look at the singer ring sign for Bronchiectasis

A
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10
Q

What are the clinical symptoms of Brochiectasis?

A

Chronic cough

Sputum production (w/ pus)

Breathlessness on exertion
Intermittent Haemoptysis
Nasal symptoms
Chest pain
Fatigue

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11
Q

What are the clinical signs of Bronchiectasis?

A

Hypoxaemia in advanced cases
Fever (recurrent)
Haemoptysis
Fine crackles (rales)
Rhonchi (low pitch snore like lung sounds)
Crackles and wheezing
Weight loss (hypercatabolic)
Clubbing

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12
Q

What are some causes of Bronchiectasis?

A

Whooping cough (pertussis), TB and measles

Immune deficiency like hypogammaglobulineemia

Mucociliarry clearance defect like Cystic Fibrosis, Youngs syndrome and Kartagener syndrome

Alpha-1-antitrypsin deficieny (loss of elastin fibres)

Obstruction
Secondary immune deficiency ((HIV and malignancy)
Rheumatoid arthritis
Idiopathic
IBS

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13
Q

What are some common causative organisms of Bronchiectasis?

A

Streptococcus pneumoniae (encapsulated)
Haemophilus influenzae (encapsulated)
Pseudomonas aeruginosa
Aspergillosis and Candida fungi
Mycobacteria Tuberculosis

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14
Q

Why is Bronchiectasis a vicious cycle if caused by infection?

A

The bronchial dilation leads to mucous accumulation, impaired ciliary faction which increases risk of infection

If further infection, more inflammation adn loss of bronchial elastic fibres and smooth muscle which leads to further dilation

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15
Q

What conditions are associated with brochiectasis?

A

Inflammatory Bowel Disease
Rheumatoid arthritis

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16
Q

What may be suggestive of a patient having Bronchiectasis?

A

Severe chest infection in early life
Life long chest infection (genetics?)
Recurrent chest infections (immmunodeficent?)
Recurrent sinus infections since child (ciliary dysfunction)?

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17
Q

If a sputum culture is taken from a patient with Bronchiectasis what organisms could be in the culture?

A

Haemophilus influenzae
Streptococcus pneumoniae
Pseudomonas’s

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18
Q

When doing spirometry to test pulmonary function, what measurements will be affected in Bronchiectasis?

A

Its an obstructive airway disease

Forced Expiratory Volume reduced and Tidal volume + residual volume increases
Inspiratory volume same

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19
Q

How can Chronic Bronchitis be differentiated from Bronchiectasis in terms of pathology?

A

Brochiectasis causes airway dilatation and scarring

Whereas chronic bronchitis causes mucus gland hyperplasia and therefore over/hyper secretion

20
Q

What are the different causes of Bronchiectasis and Chronic Bronchitis?

A

Bronchiectasis cause by persistent or severe infections

Chronic bronchitis caused by Tobacco smoke and air pollutants

21
Q

How do the symptoms of Bronchiectasis and chronic bronchitis differ?

A

Bronchiectasis = cough, purulent (smells) sputum and fever

Chronic bronchitis = cough and sputum

22
Q

How do you treat Brochiectasis?

A

Clearing the airways regularly
Sputum sampling
Exclude immunodeficiency / treat identifiable causes
Annual flu and routine vaccines against (Haemophilus influenzae and streptococcus pneumoniae)

23
Q

What is meant by an exacerbation in Bronchiectasis?

A

Person with Bronchiectasis with deterioration in 3 or more key symptoms for at least 48hrs

24
Q

What symptoms may have worsened to define an exacerbation in Bronchiectasis?

A

Cough
Sputum vol/consistency
Sputum purulence (smell)
Breathlessness
Fatigue
Haemoptysis

25
What condition can causes Bronchiectasis??
Cystic fibrosis
26
What type of inheritance is Cystic fibrosis?
Autosomal recessive
27
What is the most common mutation for Cystic fibrosis? What chromosome is affected?
Phe508del 7
28
What is the important protein that is negatively affected as a result of the Phen508del in cystic fibrosis?
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
29
What parts of the body are affected as a result of reduced expression of CFTR proteins in cystic fibrosis?
Lungs Pancreas (malabsorption) Liver (biliary cirrhosis) Sweat glands (heat shock) Vas deferens (infertility)
30
What other channels do CFTRs help regulate?
ENAC
31
What is the role of CFTR in airways?
Transports Cl- out of cells drawing out water hydrating the mucus
32
What is the most common mutation in Cystic fibrosis? How does it affect CFTR?
Phe508del Makes it less stable so they get degraded very quickly so less CFTR proteins at apical surface
33
How is CF diagnosed?
One or more of the characteristic features History of CF in sibling Positive newborn screening test result AND Inc sweat [Cl-] At least 2 CF mutations identified in genotyping
34
How does cystic fibrosis normally present?
Meconium ileus Intestinal malabsorption Recurrent chest infections Newborn screening
35
What is meconium ileus? How is it a sign of CF?
When the bowel of newborn infants is blocked by thick sticky mucus Signs of intestinal obstruction soon after birth with bilious vomiting, abdominal distension and delay in passing meconium Thick sticky mucus blocking bowel caused by the lack of CFTR activity
36
How is intestinal malabsorption (infancy) a clinical sign of CF?
Thick sticky mucus is blocking the exocrine glands of the pancreas preventing pancreatic enzymes reaching the duodenum
37
When is CF usually diagnosed?
Early in life (newborn screenings or first few years)
38
What conditions may lead you to consider underlying CF?
Recurrent idiopathic pancreatitis Recurrent sinusitis Recurrent lung infections Infertility Allergic bronchopulmonary aspergillosis
39
What are some complications of CF in the lungs?
Bronchiectasis Pneumothorax Haemoptysis Resp failure
40
What liver and gall bladder complications can happen due to Cystic Fibrosis?
Chronic liver disease Portal hypertension Gallstones
41
What are some complications of CF in the pancreas?
Diabetes Mellitus Pancreatic insufficiency
42
What are some complications of CF in the reproductive tract?
Male infertility Congenital bilateral absence of vas deferens
43
What lifestyle advice is given to patients with CF?
No smoking Avoid other CF patients (avoid pseudomonas bacteria that are antibiotic resisitant) Avoid jacuzzis (pseudomonas) Clean and dry nebulisers Annual flu vaccine NaCl tablets in hot weather or when sweating intensely
44
Why is it advised CF patients take NaCl tablets when sweating a lot?
NaCl lost in sweat so could go into heat shock
45
How is CF managed?
Holistic Strep. Pneumoniae and Haemophilus influenzae vaccines Check lung health Don’t be fat
46
What are some factors in CF contributing to impaired nutritiional status?
Pancreatic insufficiency Chronic malabsorption Chronic inflammation leading to increased energy expenditure

Y2 - Resp (23 decks)

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