Conditions Flashcards
1
Q
what does the CFTR gene control?
A
controls ion transport channels of Cl- across the cell membrane
2
Q
what happens with the mutation in the CFTR gene?
A
airway surface layer = dehydrated and cilia can no longer function properly
3
Q
summary order of CFTR mutation:
A
- CFTR gene defect
- defective ion transport
- airway surface liquid depletion
- defective MCC
- mucus obstruction
- infection + inflammation
4
Q
what does disease severity and progression depennd on?
A
o The gene type
o How well patients
are managed
5
Q
what are the main respiratory changes in CF?
A
- Submucosal gland hypertrophy
- Increased goblet cells
- Destruction of airway walls (bronchiectasis)
- Cyst formation
6
Q
how are newborns diagnosed with CF?
A
New born screening (heel prick test): if positive, then follow up sweat tests are carried out
7
Q
symptoms of CF
A
-SOB
- recurrent lung infections
- wheeze
- cough +/- sputum
8
Q
systems affected by CF
A
- Respiratory
- Endocrine
- Digestive
- Hepatobiliary
- Musculo-skeletal
- (Urinary incontinence)
- Reproductive
9
Q
how is the pancreas involved in CF?
A
- insufficient secretion of enzymes
- malabsorption of fats and some proteins
- smelly stools + deficiencies in vitamins A, D, E & K + risk of malnourishment
10
Q
how do you manage the pancreatic involvement in CF?
A
pancreatic replacement therapy
11
Q
what is CF related diabetes (CFRD) due to?
A
blocked pancreatic ducts, is a very common complication in adults with CF (15-30%) & indicates disease severity
12
Q
what is pancreatic replacement therapy?
A
aids the absorption of fats and must be taken with all meals
13
Q
what do CF patients require in a diet?
A
high in fats & protein + supplements
14
Q
how does CF affect the bile?
A
bile becomes thick and tenacious and the bile duct blocks leading to:
– cholestasis
– gall stones and
– eventually cirrhosis
15
Q
what is the acute exacerbation of CF known as?
A
bronchectasis due to CF
16
Q
bronchiectasis definition
A
the abnormal, irreversible dilation of the bronchi, where the elastic and muscular tissue is destroyed by acute/ chronic inflammation and infection
17
Q
bronchiectasis due to CF definition
A
Mutation of the CFTR gene which alters the salt concentration in the blood (controls Cl diffusion across membrane) –> excess CL in blood –> damage to MCC (dehydrated airway surface and mucous)–> mucous plugs in airways = infection & inflam
18
Q
pathology bronchiectasis
A
- permanent dilation of the bronchi and bronchioles
- loss of cilia –> squamous cells replace cilia cells
- squamous cell metaplasia
- mucous gland hyperplasia (mucous production)
- can be focal/ diffuse area
- damage to elastic and muscular tissue impairs natural drainage of bronchial secretions using Mucociliary escalator
o secretions become chronically infected
19
Q
bronchiectasis infection method
A
- initial infection
- inflammation in airways cytokines, neutrophil enzymes, bacterial products
- impaired mucociliary clearance (because of inflam)
- airway mucous hypersecretion (mucous gland hyperplasia) & obstruction (due to impaired MCC)
- mucous plug forms in airways
- microbial colonisation/ infection
- bronchial dilation/ airway destruction
- inflammation in airways etc. etc.
20
Q
bronchiectasis DUE TO CF pathogenesis
A
- CFTR dysfunction causes dysfunctional sodium channels
- Excessive sodium and water reabsorbed into the airways
- Dehydration of the airway surface and mucous
- Compresses and reduces effectiveness of cilia, impairing both MCC and cough effectiveness
- Increased frequency chest infections
21
Q
what does a contrast-enhanced CT show for bronchiectasis?
A
grossly dilated airways & cystic changes
22
Q
bronchiectasis causes
A
- congenital conditions with MC dysfunction
e.g. primary cilial dyskinesia (PCD), CF - airway obstruction & then irritation & infection
- inherited genetic disease
- autosomal recessive pattern 1:4 change of inheriting if both parents are carriers of gene
- thick tenacious mucous
- COPD & Asthma
- allergy, aspiration
23
Q
bronchectasis causes summary
A
- CF
- mucous in airways that cant be cleared so it gets infected
- foreign bodies in airways that cant be cleared so causes infection and distruction of bronchiole walls etc.
24
Q
signs of CF
A
- cough with excessive sputum (>30 mL/day)
–> loose cough
–> purulent secretion
–> +/- haemoptysis (due to inflamed airway mucous)
–> thick, tenacious sputum - reports recurrent exacerbations / infections needing antibiotics (patients with >3 exacerbations/year)
- dyspnoea
- fatigue
- recurrent pulmonary infections
- execise intolerance
- R sided heart failure
25
symptoms of CF
- chronic cough
- thick tenacious sputum
- recurrent pulm infections
- recurrent eps of bronchitis, pneumonia, bronchiectasis
- exercise intolerance
-
26
bronchiectasis due to CF chest X-Ray
- Thickening of bronchial walls
- Irregular shaped bronchioles
- Mucous plugging
- Widespread ‘patchy’ opacities/ mucoid impaction
27
bronchectasis due to CF ABG
- Extensive disease may result in hypoxaemia
- Raised PaCO2 = rare
28
CF bronchiectasis PFT
Obstructive Pattern
- FVC decreased
- FEV1 decreased
- TLC normal
- DLCO normal
- FEV1/FVC <70% predicted
29
CF bronchiectasis auscultation
variable
- coarse crackles on inspiration & expiration (mucous in airways)
- wheeze (narrowed airways)
30
IMPAIRMENTS DUE TO CF BRONCHIECTASIS
secretion movement impairment (MCC)
decreased MCC
- dehydration of the airway surface (CFTR gene mutation) = destroys cilia
- primary disruption to the airway lining (inflammation) affects MCC
- decreased MCC = mucous plugging = infection of mucous = damage to the bronchioles = bronchiectasis
31
what does destruction and dilation of airways cause?
'floppy airways' (obstruction on expiration)
32
what does chronic CF infection cause?
fibrosis of lung parenchyma
33
what does fibrosis of lung parenchyma disrupt?
O2 movement across membrane
34
IMPAIRMENTS DUE TO CF BRONCHIECTASIS
Secretion movement (cough)
- airflow limitation (cant get air out during expiration) -- cant get air behind cough = ineffective cough
- thick mucous plugs in airways + ineffective cough (due to lack of O2/ floppy airways (cant get air out)) = obstruction
35
what happens to the airways in CF bronchiectasis
floppy and fibrosed (due to recurrent infections)
36
what does CF bronchiectasis damage?
- lung parenchyma
- lung compliance (decreases lung compliance)
37
IMPAIRMENTS DUE TO CF BRONCHIECTASIS
Gas movement (O2)
permanent scarring of lung parenchyma, due to chronic infection, decreases O2 movement across airway walls (also as they are fibrosed & floppy - reduced SA)
38
IMPAIRMENTS DUE TO CF BRONCHIECTASIS
Pump effectiveness (e.g. NMC, muscles, bones)
decreased pump effectiveness
39
IMPAIRMENTS DUE TO CF BRONCHIECTASIS
Gas movement (CO2)
decreased CO2 movement as floppy airways disable the air (CO2) from coming out of body on expiration
40
NON CF bronchiectasis definitoin
progressive condition with irreversible destruction & dilation of airways
41
pathology of non CF bronchiectasis
- chronic inflammatory changes
- damage to airway structures
- dilation of airways
42
Non CF bronchiectasis pathogenesis
- Recurrent infection/ inflammation damage bronchi and bronchioles
- Loss of elastic and muscular tissue (due to repeated infection) = dilation of bronchi
- Loss of cilia impairs MCC
- Remaining bacteria remains in airways and colonise mucous
- Dilated ‘floppy’ airways = airflow obstruction
- Cilia dysfunction and mucous production cause this cycle to repeat causing disease progression
- Excessive sputum production (mucous gland hyperplasia) and impairment to MCC = airflow limitation
43
non CF bronchiectasis signs & symptoms
- Chronic productive cough--> sputum production
- Copious amounts of purulent, foul-smelling, green or yellow sputum
- Haemoptysis sometimes
- Dyspnoea --> due to airflow limitation on expiration (O2 movement is impaired as it cant diffuse across membrane walls easily as they = inflamed, thick, swollen, mucous plugging
- digital clubbing
44
non CF bronchiectasis auscultation
coarse crackles on inspiration and expiration (mucous plugging and mucous in airways)
45
non CF bronchiectasis CXR findings
- Thickening of bronchial walls
- Irregular shaped bronchioles
- Mucous plugging
* HRCT scan is gold standard
46
Non CF bronchiectasis ABG
- extensive disease may result in hypoxemia
- raised PaCO2 = rare
47
non CF bronchiectasis PFT
obstructive pattern
- decreased FVC
- TLC normal
- decreased FEV1
- DLCO normal
- FEV1/ FVC <70%
48
IMPAIRMENTS OF NON CF BRONCHIECTASIS
Secretion movement (MCC + cough)
decreased MCC (due to destruction of airway walls?) --> secretion movement issue --> ineffective cough
49
IMPAIRMENTS OF NON CF BRONCHIECTASIS
Gas movement (O2)
destruction & dilation of airways causes 'floppy' airways & scarring of lung parenchyma
--> disrupts O2 movement across membrane (reduced SA + destruction of airway walls)
50
COPD
Chronic Bronchitis definition
expectoration of COPD on most days for at least 3 months in the year for at least 2 consecutive years
51
chronic bronchitis pathology
- mucous gland hypertrophy--> increased mucous production & decreased clearance
- structure of the airways is damaged --> damage to the secretion movement of the airways e.g. mucociliary clearance
- cilial dysfunction --> cant clear mucous from airways
- chronic inflammation of small airways --> narrowing, cellular infiltration, airway wall oedema
52
chronic bronchitis symptoms
- increased mucous production
- increased sputum production (cough +/- sputum)
- shortness of breath
- abnormal pattern of breathing
53
chronic bronchitis signs
- increased sputum production
- abnormal pattern of breathing
- hyperinflated lungs
54
chronic bronchitis auscultation
reduced breath sounds, coarse crackles
55
chronic bronchitis PFT
- Decrease FEV1/FVC
- decrease FEV1 %
obstructive pattern
56
chronic bronchitis ABG
PaCO2 increases and PaO2 decreases, SpO2 decreases
57
why is bone disease common in CF?
they have low BMD
58
why do CF patients have low BMD ?
decreased rates of bone accretion, and increased rate of bone loss
59
how do you MAXIMISE peak bone mass in adolescents?
* Regular WB exercises (plyometric – jump/hop/skip etc)
* Strength and resistance training
60
what do adults with CF have with bones?
accelerated bone loss
61
how can you maintain bone mass & slow the rate of loss in adults with CF?
* Exercise (WB, PA/ resistance training)
* Diet
* Medications
62
what are the fracture rates like in the CF population?
high
63
what can fractures in CF patients contribute to?
throacic kyphosis
64
why is screening for M/S pain (acute and chronic) a key aspect of CF care?
patients live longer
65
how does CF affect chest shape?
hyperinflated chest --> tight postural muscles & pecs
66
what movements does CF limit?
shoulder and neck flexion
67
what postural position does CF cause ?
Thoracic kyphosis
68
why are females with CF encouraged to reach optimum nutrition and lung function before pregnancy?
– to aid conception
– to minimise the detrimental effects pregnancy will have on these symptoms
69
potential causes of incontinence in CF:
– Chronic cough or paroxysms of prolonged coughing
– Demands placed on the pelvic floor during airway clearance therapy, (eg, huffing, coughing & exercise)
– Underlying structural differences
70
CF Rx methods
* Prevent acquisition of bacteria (prevents bronchiectasis --> chronic infection of the clogged mucous)
* Strict infection control ( Staff and patients)
* Isolation of inpatients
* Pathogen specific clinics
* Discourage socialisation (between those with CF)
except in well ventilated large open spaces
* Airway clearance techniques
* *Modulator therapies
- antibiotics
71
purpose of antibiotics in CF
Attempt to eradicate bacteria on first presentation with aggressive therapy combinations
72
different ways to insert antibiotics into blood
IV, inhaled or oral
* In hospital or IV at home
* IV therapy via
– Peripherally inserted central line (PICC) or
– Portacath
73
how is hypertonic saline used as a Rx for CF?
The body tries to dilute this strong salt solution by the
release of water into the airways
74
what happens to the secretions after inhaling hypertonic saline?
The secretions become less viscous & are therefore more
easily expectorated
75
wht can hypertonic saline induce?
bronchospasm in clients with reactive airways (adminster with ventolin)
76
how is dornase alfa (pulmozyme) administered?
via nebuliser in people with CF who have thick viscous secretions containing decaying inflammatory cell and bacteria
77
what is pulmozyme
Recombinant human deoxynuclease
78
how does pulmozyme work?
Cleaves long strands of DNA into smaller strands and
thereby “liquefies” sputum making it easier to clear
79
what type of agent is mannitol?
Osmotic agent
80
how does Mannitol help in CF?
Decreases viscosity of secretions
81
Rx regimens in CF:
- Antibiotic treatments (oral
or intravenous): 80—95%
- Nebulised medicines and
pancreatic enzymes: 65—
80%
- Vitamin therapy, dietary
management, exercise
regimens and physiotherapy
airway clearance: 40—55%
82
how does a decreased MCC in CF create a secretion movement problem?
cilia = ineffective in clearing mucous (they are dried out due to the airway wall dehydration) = recurrent infections and further cilial damage
83
how is the mucous a secretion movement factor in CF?
viscosity increases (thicker & stickier) which then causes the cilia to be ineffective
84
how does CF affect airways?
recurrent infections lead to floppy airways (damaged & fibrosed)
85
how does CF affect lung parenchyma?
recurrent infection leads to damage (fibrosis) of alveoli, interstitium, capillaries
86
how does CF cause a weak/ ineffective cough?
obstruction on expiration = decreased EFR = decreased volition = decreased cough effectiveness
87
what happens to the inspired volumes in CF?
decreased
88
what happens to lung compliance in CF?
decreased
89
respiratory load in CF?
increased
90
Pneumonia definition
An acute inflammatory condition of the lung parenchyma
91
pneumonia pathology
Alveolar spaces are filled partially or completely with fluid and blood cells (infectious debris & exudate)
92
how can pneumonia be classified?
1. site of infection
2. source
3. cause
93
pneumonia classification by site of infection
* Lobar pneumonia
* Bronchopneumonia
94
pneumonia classification by source of infection
* Community acquired pneumonia (CAP)
* Nosocomial / hospital acquired
* Ventilated acquired pneumonia (VAP)
95
pneumonia classification by cause of infection
aspiration pneumonia, pneumocystis carinii pneumonia (fungi)
96
on the CXR, what are the telltale features of lobar pneumonia?
- consolidation
- no loss of lobe vol
- air bronchograms
97
on the CXR, what are the telltale features of bronchopneumonia?
patchy distribution of consolidation
- air bronchograms (air trapped in airways)
98
risk factors of pneumonia
- stroke
- poor nutrition
- smoking
- alcoholism
- winter
- extremes of age
99
PATHOGENESIS OF PNEUMONIA
Stage 1: hyperaemia/ congestion
acute inflammatory response (first 24 hours)
100
PATHOGENESIS OF PNEUMONIA
Stage 2: red hepatisation (2-3 days)
alveoli fill with RBC, exudate, and fibrin
101
PATHOGENESIS OF PNEUMONIA
Stage 3: grey hepatisation (4-8 days)
WBC colonise area and phagocytosis of cell debris occurs
102
PATHOGENESIS OF PNEUMONIA
Stage 4: resolution (8days -weeks)
immune & inflammatory responses decrease, macrophages clearn up area
103
what happens to Pneumonia in terms of obstructive & restrictive?
starts as obstructive AF limitation gas mvmt impairment due to increased distance of exchange additional inflammation leads to restrictive airflow changes
104
signs/symptoms of Pneumonia
– Cough ± sputum/haemoptysis
– ± Chest wall (pleuritic) pain
– Changed pattern of breathing
105
ausc of pneumonia
- wheezing
- decreased BS
- fine crackles (alv opening to get more air)
106
aspiration definition
inhalation of either oropharyngeal or gastric contents into the lower airways
107
what is aspiration pneumonia
aspiration of bacteria from
oral and pharyngeal areas
108
what can aspiration pneumonia result in?
– Purulent sputum
– Tissue necrosis
– Pulmonary cavities (anaerobic lung abscess)
109
pneumonia CXR & ABG
– CXR: infiltrate/opacity
– ABG changes (reduced PaO2)
110
Pneumonia constitutional symptoms (indicate acute infection)
– Fever
– Myalgia
– Malaise
– Tachycardia
111
how does pneumonia affect secretion movement?
infection causes cilial dysfunction and loss (inability to move secretions), some pneumonias will have mucus
112
what happens to the alveoli in pneumonia?
infection and its by-products fill the alveoli
113
how does Pneumonia cause an O2 movement problem?
filling of the alveoli with exudate decreases surface area and decreases O2 diffusion across membrane
114
is lung compliance increased or decreased in pneumonia?
decreased
115
how can a decreased O2 movement affect the cough?
- decrease volition
- decrease size of breath in
- decrease cough effort
(cant get big breath behind it)
116
how does pneumonia affect muscles?
inflammation of the pleura causes pain --> impacts muscle function
117
medical management of pneumonia
- antibiotics
- other drugs to decrease airway inflamm
- supportive therapies e.g. o2 therapy
- physio management e.g. AC techs
118
why do indigenous australians have a higher incidence of bronchiectasis?
linked to
poorer health and lack of antibiotic use
119
causes of bronchiectasis
1. congenital conditions with MC dysfunction
2. immune deficiencies
3. Airway obstruction (inhalation/tumour)
4. Aspiration (reflux)
5. Allergic bronchopulmonary aspergillosis (ABPA)
6. COPD and Asthma: numbers underreported
7. Idiopathic Bronchiectasis associated with NTM
120
signs & symptoms of bronchiectasis
- Cough with excessive sputum (>30ml/day)
- Reports recurrent exacerbations
- Dyspnoea
- fatigue
121
bronchiectasis auscultation s
variable e.g. coarse crackles (sputum in airways), wheeze
122
bronchiectasis spirometry
mixed pattern
123
bronchiectasis CXR/CT scan
HRCT scan better for diagnosis &
monitoring progression than CXR
124
how does bronchiectasis affect MCC?
infection causes disruption to airway lining: MCC, specifically cilia
- infection causes loss of cilia
125
how does a decreased MCC cause a secretion movement problem?
long term effects of decreased MCC = chronic infection (mucus) and damage to airway
- cant clear mucus due to loss of cilia - mucus clogs airays = infected
126
what happens to the airways in bronchiectasis?
destruction & remodeling
127
how does bronchiectasis effect cough?
obstruction on expiration decreased EFR
128
how does bronchiectasis affect airway resistance?
destruction and dilation causes 'floppy' airways (obstruction)
- increases airway resistance
129
how does chronic infection affect the parenchyma in bronchiectasis?
results in fibrosis of lung parenchyma
130
goals of Mx of bronchiectasis
to optimise:
- General well-being
- Symptom control
- Quality of life (QoL)
- Reduce exacerbation frequency
- Prevent excessive decline in lung function
131
stepwise Rx approach of symptomatic BE
1. treat underlying cause e.g. foreign body
2. optimise airway clearance (e.g. techniques), inhaled mucokinetics
3. treat infection (acute and chronic)
- oral antibiotics (10days)
- IV antibiotics (5-14 days)
132
what does a restrictive disorder do on inspiration?
Prevents adequate volume entering the lungs
133
what happens to FVC and FEV1 with restrictive disorders?
decreases (ratio is normal or slightly increased)
134
tidal volume wih restrictive disorders
decreased ( with compensatory increased RR)
135
examples of disorders causing restriction
* Neurological injury or disease below the brainstem
* Weak respiratory muscles
* Skeletal chest wall is stiff or too floppy
* Disorders of the pleural space
* Stiff lungs
136
what do restrictive disorders generally affect?
the respiratory pump e.g. muscle, pleura, skeletal system, NM
137
types of NM disorders that cause restriction
- Transmission of signals to muscles is reduced/absent
- Neuropathies
- Neuromuscular junction abnormalities
- Muscle diseases
138
POB with restrictive disorders
decrease VT, decrease RR, may use different muscles to
breathe, dependent on condition
139
restrictive disorders auscultation
decreased breath sounds
140
ABG restrictive disorders
decrease oxygenation (PaO2)
141
what characterises a restrictive disorder?
- diminished lung volume
- decreased TLC, FVC
- normal or increased FEV1/FVC ratio
142
what causes pump failure in restrictive disorders?
- neural / primary muscular causes
143
does restrictive disorders cause gas movement issues?
yes
144
restrictive disorders and CO2 gas movement impairments
decreased gas movement if prolonged disease/ severe enough
145
restrictive disorders and O2 gas movement impairments
- decreased FRC
- decreased breath size (cant get enough air in)
146
how do restrictive disorders affect cough effectiveness?
decreases
- if inspiratory volume inadequate (cant get air in to cough)
- +/- expiratory muscles affected
147
compliance definition
ease with which the lungs and chest wall can be expanded during inspiration
148
which areas of the lung have a low compliance?
top (need more pressure to get air in)
149
which area of the lung has higher compliance?
bottom (more dependent regions of the lung)
149
what does reduced compliance mean?
the transpulmonary pressure
must change by a greater amount for a given volume
– ie, The lung/chest wall is harder to move/stiffer
– There is an increased elastic work of breathing
149
what can the respiratory system compliance be affected by?
changes to either:
– lung compliance (elastic recoil, surfactant changes)
and/or
– chest wall compliance (joints, ligaments, muscle changes)
150
skeletal disorders of the chest wall
– Scoliosis/kyphosis (alter joint mechanics/mm function)
– Ankylosing spondylitis (causes fibrosis and ossification of joints, including CV joints)
– Obesity (increased load from excessive soft tissue)
– Flail chest (loss of chest wall rigidity)
150
obesity effect on cardiorespiratory system
– Reduces lung volumes, particularly ERV & FRC
– Reduces effectiveness, strength and endurance of respiratory muscles
– Increases respiratory load & effort (WOB)
150
Respiratory signs and symptoms of restrictive CW disorders:
spirometry
restrictive pattern
151
Respiratory signs and symptoms of restrictive CW disorders:
Changed POB
decreased VT, increased RR, asymmetrical (eg if scoliosis)
152
Respiratory signs and symptoms of restrictive CW disorders:
auscultation
decreased breath sounds, may be asymmetrical
153
Respiratory signs and symptoms of restrictive CW disorders:
ABG
decreased oxygenation
154
chest wall compliance with scoliosis, ankylosing spondy, obesity
chest wall stiffer, therefore harder to expand
--> decreases compliance
155
chest wall compliance with flail chest
Chest wall floppier
(paradoxical segment)
therefore less expansion for
same muscle effort
156
DISORDERS OF THE PLEURA/ PLEURAL SPACE
– Pneumothorax
– Fluid in the pleural space:
effusion/haemothorax/empyema/chylothorax
– Pleural thickening
157
Pneumothorax definition
air in the pleural space (i.e. between the visceral and parietal pleura)
158
pneumothorax pathology
Loss of negative intrapleural pressure causes the lung
to collapse inwards
159
what happens to the pleura layers in a pneumothorax
o visceral pleura separates from parietal, air enters IPS, air pushes/ collapses the lung down
160
pneumothorax aetiology
Spontaneous/ primary
o Unknown cause
Secondary
o Rupture of alveoli, bulla, or blebs
Traumatic
o Penetrating injuries of the chest
o Fractured rib
o Consequence of medical emergencies
161
pneumothorax symptoms
- racing heart (tachycardia)
- sudden chest pain on affected side
- dyspnoea (increased WOB)
162
pneumothorax signs
- ABG decreased PaO2 (not routinely taken)
- Dyspnoea
- Spirometry = not done for acute conditions
- Changed POB (muscles cant do the same amount of expansion that would normally occur at chest wall; due to decreased chest wall compliance, the POB changes)
- Changed POB (may be asymmetrical)
163
pneumothorax auscultation
- Decreased/ absent breath sounds
- pleural rub
- bronchial breath sounds
164
why arent PFT's routinely taken acutely?
not a restrictive/ obstructive disorder --> disorder of chest wall
165
CXR pneumothorax
- increased lucency
- lung edge visible
- absence of lung markings
166
pneumothorax impairments
- restrictive airflow limitation
- gas movement impairment (as the air outside the lung does not participate in the exchange)
- decreased lung compliance
- decreased chest wall compliance
- impairments of the respiratory pump
