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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
Q

What condition can causes Bronchiectasis??

A

Cystic fibrosis

26
Q

What type of inheritance is Cystic fibrosis?

A

Autosomal recessive

27
Q

What is the most common mutation for Cystic fibrosis?

What chromosome is affected?

A

Phe508del

7

28
Q

What is the important protein that is negatively affected as a result of the Phen508del in cystic fibrosis?

A

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

29
Q

What parts of the body are affected as a result of reduced expression of CFTR proteins in cystic fibrosis?

A

Lungs
Pancreas (malabsorption)
Liver (biliary cirrhosis)
Sweat glands (heat shock)
Vas deferens (infertility)

30
Q

What other channels do CFTRs help regulate?

A

ENAC

31
Q

What is the role of CFTR in airways?

A

Transports Cl- out of cells drawing out water hydrating the mucus

32
Q

What is the most common mutation in Cystic fibrosis?
How does it affect CFTR?

A

Phe508del

Makes it less stable so they get degraded very quickly so less CFTR proteins at apical surface

33
Q

How is CF diagnosed?

A

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
Q

How does cystic fibrosis normally present?

A

Meconium ileus
Intestinal malabsorption
Recurrent chest infections
Newborn screening

35
Q

What is meconium ileus?

How is it a sign of CF?

A

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
Q

How is intestinal malabsorption (infancy) a clinical sign of CF?

A

Thick sticky mucus is blocking the exocrine glands of the pancreas preventing pancreatic enzymes reaching the duodenum

37
Q

When is CF usually diagnosed?

A

Early in life (newborn screenings or first few years)

38
Q

What conditions may lead you to consider underlying CF?

A

Recurrent idiopathic pancreatitis
Recurrent sinusitis
Recurrent lung infections
Infertility
Allergic bronchopulmonary aspergillosis

39
Q

What are some complications of CF in the lungs?

A

Bronchiectasis
Pneumothorax
Haemoptysis
Resp failure

40
Q

What liver and gall bladder complications can happen due to Cystic Fibrosis?

A

Chronic liver disease
Portal hypertension

Gallstones

41
Q

What are some complications of CF in the pancreas?

A

Diabetes Mellitus
Pancreatic insufficiency

42
Q

What are some complications of CF in the reproductive tract?

A

Male infertility

Congenital bilateral absence of vas deferens

43
Q

What lifestyle advice is given to patients with CF?

A

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
Q

Why is it advised CF patients take NaCl tablets when sweating a lot?

A

NaCl lost in sweat so could go into heat shock

45
Q

How is CF managed?

A

Holistic
Strep. Pneumoniae and Haemophilus influenzae vaccines
Check lung health
Don’t be fat

46
Q

What are some factors in CF contributing to impaired nutritiional status?

A

Pancreatic insufficiency
Chronic malabsorption
Chronic inflammation leading to increased energy expenditure

Resp (23 decks)

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