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Pulm > Lec 12 Bronchiectasis > Flashcards

Lec 12 Bronchiectasis Flashcards

1
Q

What is the definition of bronchiectasis?

A

permanent abnormal dilation of bronchi

pathologic/radiological diagnosis

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2
Q

What is vicious cycle hypothesis of bronchiectasis?

A

external insult –> causes resp tract damage –> more prone to infection –> more inflammation –> more damage etc

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3
Q

What are infectious causes of bronchiectasis?

A

measles/pertussis pneumonia

other bacteria/viruses –> TB, mycobacterium

allergic bronchopulmonary aspergillosis

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4
Q

Who gets allergic bronchopulmonary aspergillosis?

A

patients with asthma or cystic fibrosis

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5
Q

What are some causes of recurrent airway infection?

A
  • airway obstruction
  • impaired mucous clearance
  • defect in host defense
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6
Q

What are the 3 patterns of bronchiectasis?

A
  • cylindrical = smooth dilation of bronchus
  • varicose = focal narrowings along a dilated bronchus
  • cystic = progressive dilation of bronchus which terminates in cysts or saccules
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7
Q

What signs of bronchiectasis?

A 
permanently dilated airways
cough
purulent sputum
recurrent infections
hemoptysis
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8
Q

What causes hemoptysis in bronchiectasis?

A

inflammation causes increased blood supply

  • bronchial arteries enlarge and increase in number; new anastamoses form between bronchial + pulm artery circulations

erosion/truama leads to rupture/hemoptysis

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9
Q

What do you see on physical exam with bronchiectasis?

A
  • may have normal lugn exam or wheezing

- clubbing

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10
Q

What do you see on pulm function tests with bronchiectasis?

A

low FEV1/FVC

high RV and RV/TLC ratio

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11
Q

What is treatment for bronchiectasis?

A
  • antibiotics with exacerbation
  • bronchopulmonary drainage
  • bronchodilators if co-existing asthma
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12
Q

What is epidemiology of CF?

A
  • autosomal recessive
  • mutation in gene for cystic fibrosis transmembrane conductance regulator [CFTR]
  • most prevalent in pple of northern/central europe descent
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13
Q

What is function of CFTR?

A
  • ion channel regulates Cl and H2O absorption/secretion

in sweat duct = Na but not fluid absorbing
in airways = fluid absorption
in pancreas/intestine = fluid secretion

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14
Q

What happens in lungs in CF?

A
  • lung epithelium dried out = cilia can’t move as well and stuff gets stuck
  • less airway surface liquid volume

normally when high liquid volume: channels open allow Na/Cl absorption and H2O to follow in but close when low volume and net water out

in CF = can’t regulate; channels always open and bring fluid in even when there is already low volume

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15
Q

What happens in CF in pancreas?

A
  • CFTR is needed to secrete HCO3 and water into pancreatic ducts

in CF –> acidic secretions lead to retention of enzymes in pancreas and destruction of pancreatic tissue

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16
Q

What happens in CF in intestine?

A

intestinal epithelium can’t flush secreted mucin and other macromolecules

have intestinal obstruction

17
Q

What happens in CT in sweat glands?

A
  • CFTR needed to reabsorb Cl from sweat secretions

in CF –> Cl not reabsorbed have isotonic [rather than hypotonic] sweat

18
Q

What are the 6 types of CFTR mutations?

A 
type 1 = no functional CFTR protein
type 2 = CFTR trafficking defect, never reaches surface
type 3 = defective channel regulation
type 4 = decreased channel conductance
type 5 = reduced synthesis of CFTR
type 6 = decreased CFTR stability
19
Q

What is type 2 CFTR mutation?

A

normal transcription but abnormal folding –> have trafficking defect and CFTR never makes it to cell surface

20
Q

What is type 1 CFTR mutation?

A

transcription abnormal = no protein produced

21
Q

What is type 3 CFTR mutation?

A

transcription/translation normal but abnormal regulation

22
Q

What is type 4 CFTR mutation?

A

decreased conductance of chloride in channel

23
Q

What is type 5 CFTR mutation?

A

have some corent + some incorrect RNA due to splicing defect

so end up with reduced synthesis of CFTR

24
Q

What is type 6 CFTR mutation?

A

have decreased CFTR membrane stability

25
Q

What is major mutation causeing 70% of CF?

A

F508 mutation

  • 3 nt deletion
  • causes protein misfolding = class II defect
26
Q

What doe you always have if homozygous dF508 mutation?

A

always have exocrine pancreatic insufficiency

27
Q

What is pulmonary pathology of CF?

A
  • thick mucous plugs in bronchi

- early airway changes mostly in bronchioles plugged by secretions

28
Q

What is pulmonary pathophysiology of CF?

A
  • recurrent episodes of tracheobronchial infection
  • leads to bronchiectasis
  • get pseudomonas + MRSA colonization
  • get obstructive airway disease
29
Q

What are presenting features of CF?

A
  • 10-20% develop first clinical problem in neonatal period –> usually meconium ileus = obstructed intestine
  • rest have childhood presentation –> pancreatic insufficiency or recurrent bronchial infections
  • occasionally diagnosed when adults
30
Q

What are pulmonary complications of CF?

A
  • pneumothorax
  • hemoptysis
  • allergic bronchopulmonary aspergillosis
  • pulmonary HTN
  • resp failure
31
Q

What are non-pulmonary clinical features of F?

A
  • nasal polyps
  • chrnoic pancreatisis
  • intestinal obstruction
  • exocrine pancreas insuffiency [malabsorption of vit A, D, E, K]
  • rectal prolapse
  • infertility in males
32
Q

How do you diagnose CF?

A
  • abnormal sweat electrolytes [high Na, Cl, K]

- identify mutation in CFTR gene

33
Q

What is treatment for CF?

A

manage complications

for lungs: antibiotics, bronchopulmonary drainage, bronchodilators; give N-acetylcysteine to loosen mucus plugs

34
Q

What is use of inhaled recombinant doexyribonuclease in CF?

A
  • DNAse decreases mucus viscosity + improves clearance of secretions
35
Q

What is ivacaftor?

A

increases time activated CFTR channels at cell surface remain open

improves respiratory symptoms in pts with G511D mutation

36
Q

What is primary ciliary dyskinesia?

A

autosomal recessive group of structural deficits involving cilia

37
Q

What is kartagener’s syndrome?

A
  • autosomal recessive ciliary dyskinesia syndrome
  • characterized by
  • — bronchiectasis,
  • — sinusitis
  • —- situs inversus [in 50%]

Pulm (24 decks)

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