Respiratory Flashcards

1
Q

What does FVC stand for in lung function testing?

A

Forced Vital Capacity

Total volume exhaled, not total lung volume.

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2
Q

What is the significance of a FEV/FVC ratio of <0.70?

A

Indicates airflow limitation pattern (asthma, COPD)

This ratio is used in spirometry to assess lung function.

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3
Q

What does TLC stand for?

A

Total Lung Capacity

A reduction (<80%) is seen in restrictive diseases.

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4
Q

What is the definition of Residual Volume (RV)?

A

Volume of air remaining in the lungs after maximal exhalation

Elevated with gas trapping, typically seen in COPD.

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5
Q

What is FENO and its significance?

A

Fraction expired nitric oxide

Indicates airway eosinophilia and is a marker of asthma activity.

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6
Q

What does a positive bronchial challenge test indicate?

A

A fall in FEV1 of 20% or more with serial spirometry measurements

Normal results are the gold standard for asthma exclusion.

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7
Q

What is the normal A-a gradient for a young adult?

A

5-10 mm Hg

The upper limit of the A-a gradient rises by 1 mmHg per decade of life.

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8
Q

What is Type 1 Respiratory Failure characterized by?

A

Reduced oxygenation with a reduction in pO2 and normal pCO2

Often associated with acute asthma.

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9
Q

What imaging modality is preferred for suspected interstitial lung disease?

A

High Resolution Computed Tomography (HRCT)

Provides ultra-thin slices for higher resolution imaging.

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10
Q

What is the purpose of a Ventilation-Perfusion (VQ) Scan?

A

To assess for pulmonary embolism by comparing ventilation to perfusion

It is highly regarded for assessing CTEPH.

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11
Q

What does the Oxygen Dissociation Curve illustrate?

A

The capacity of hemoglobin to release oxygen

It shifts with varying temperatures, DPG concentrations, and CO2 partial pressures.

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12
Q

What is the role of bronchoscopy in respiratory assessment?

A

To visualize tracheal or bronchial lesions and collect respiratory samples

It is a safe procedure with a low mortality rate.

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13
Q

What characterizes the early phase response in asthma?

A

Bronchoconstriction and airway obstruction occurring 15-30 mins after allergen exposure

Resolves within 2 hours.

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14
Q

What is the significance of elevated fraction expired nitric oxide (FENO) in asthma?

A

Indicates active airway eosinophilia

Expected to be high at the time of asthma diagnosis without treatment.

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15
Q

What is the main purpose of the Six Minute Walk Test?

A

To assess a patient’s exercise capacity

Used prior to and following pulmonary rehabilitation.

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16
Q

What is the definition of Diffusion Capacity of Carbon Monoxide (DLCO)?

A

Reflects the ability of diffusion from alveolar gas to erythrocytes

Increased DLCO indicates asthma or pulmonary hemorrhage, while decreased indicates COPD or restrictive lung diseases.

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17
Q

What is one common cause of a raised A-a gradient?

A

V/Q mismatch

Can occur with conditions like pneumonia or pulmonary embolism.

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18
Q

What does spirometry reveal in a patient with asthma?

A

FEV1/FVC <0.70 and an increase in FEV1 after bronchodilator therapy

It is critical for diagnosing asthma.

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19
Q

What is the definition of Type 2 Respiratory Failure?

A

Reduced oxygenation with a marked increase in pCO2

Often leads to respiratory acidosis.

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20
Q

What is the expected DLCO in a patient with COPD?

A

Decreased DLCO

This reflects impaired gas exchange.

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21
Q

What does corrected diffusion capacity (KCO) reflect?

A

DLCO in relation to the surface area available for diffusion

Increased KCO suggests extrapulmonary restriction.

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22
Q

What is an important consideration when documenting intercostal catheter (ICC) output?

A

Volume removed and type of fluid

Removing >1L per hour may predispose to re-expansion pulmonary edema.

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23
Q

What role does allergen testing play in asthma diagnosis?

A

Determines allergen sensitivity

Skin prick testing is first-line; RAST can determine serum IgE levels.

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24
Q

What is chronic phase asthma characterized by?

A

Chronic sub-epithelial fibrosis and smooth muscle cell hypertrophy

Results from uncontrolled inflammation and remodelling.

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25
What is the significance of the arterial blood gas (ABG) in respiratory assessment?
Provides critical information on pH, pO2, pCO2, HCO3-, and Base Excess ## Footnote Essential for assessing acid-base balance and respiratory function.
26
What does exhaled nitric oxide testing relate to?
Airway eosinophilia and active inflammation ## Footnote Levels are decreased with corticosteroid use and can be used to determine both treatment effectiveness and adherence.
27
What is the first line for allergen sensitivity testing?
Skin prick testing ## Footnote Radioallergosorbent Testing (RAST) can also be performed to determine serum IgE levels to some allergens.
28
What role does total IgE play in eosinophilic phenotype?
It has some role but is not diagnostic.
29
When are IgE levels particularly significant?
When considering biological therapy for severe, refractory cases of asthma.
30
What is the stepwise guide to classical asthma management?
1. SABA prn 2. Low dose ICS 3. Low dose ICS + LABA 4. High dose ICS + LABA 5. High dose ICS + LABA + systemic corticosteroid 6. High dose ICS + LABA + biological therapy
31
What does SABA stand for?
Short acting beta-agonist.
32
What is the role of SABA in asthma management?
Available for all patients as prn therapy and can be used prior to exercise.
33
True or False: LABA monotherapy is appropriate in eosinophilic asthma.
False.
34
What is the mainstay of therapy for preventing exacerbations in asthma?
Inhaled corticosteroids (ICS).
35
What are some significant side effects of ICS?
* Oral, laryngeal, and oesophageal candidiasis * Hoarse voice * Cough * Potential chronic systemic side effects - bruising, osteopenia, adrenal suppression, weight gain.
36
What is the optimal daily dose of ICS?
600 mcg/day.
37
What is the primary indication for systemic corticosteroids?
In the acute setting, they are very effective.
38
What do leukotriene modifiers do?
They block leukotriene receptors, promoting reduction in mucous secretion, vasodilation, and inflammation.
39
What are some side effects of leukotriene modifiers?
* Agitation * Anxiety * Hallucinations * Suicidal ideation * Rarely, hepatotoxicity.
40
What is the mechanism of action of Omalizumab?
It binds IgE, preventing the binding and activation of mast cells.
41
What is the indication for Mepolizumab?
Treatment resistant eosinophilic asthma on maximal medical therapy.
42
What is the 'Sumter Triad' associated with?
Severe asthma, aspirin sensitivity, and nasal polyps.
43
What is the primary treatment for allergic bronchopulmonary aspergillosis (ABPA)?
Immunosuppression.
44
What does the GOLD classification of COPD severity use to define airflow limitation?
FEV1/FVC < 70%.
45
What is the BODE Index used for?
To decide if a patient is suitable for lung transplantation.
46
What is the first step in the pharmacological management of COPD?
SABA as required for mild disease (FEV1 > 60%).
47
What is the role of inhaled corticosteroids (ICS) in COPD?
ICS should be reserved for symptomatic patients on LABA + LAMA with frequent exacerbations.
48
What is the risk associated with the long-term use of ICS in COPD?
Increased risk of pneumonia in the elderly.
49
What is the therapeutic window of Theophylline?
Narrow.
50
What is a key characteristic of occupational asthma?
Patients often describe symptom-free periods away from work.
51
What are common adverse outcomes from poorly controlled asthma in pregnancy?
* Low birth weight * Pre-eclampsia * Premature labor * Increased infant mortality.
52
What is the role of Inhaled Corticosteroids (ICS) in COPD?
ICS should not be used as monotherapy in COPD and are reserved for symptomatic patients on LABA + LA?/IA with frequent exacerbations. ## Footnote ICS have shown further benefit as monotherapy over inhaled corticosteroids.
53
What is the risk associated with ICS in elderly patients?
There is a significantly increased risk of pneumonia in the elderly. ## Footnote This was highlighted in the TOPCH Study.
54
What is the indication for Theophylline in COPD management?
Indicated in severe, difficult to manage COPD, but rarely used due to a narrow therapeutic window and potential toxicity. ## Footnote The exact mechanism of action is unclear, but may involve phosphodiesterase inhibition.
55
What is the primary effect of PD4 inhibitors like Roflumilast?
Roflumilast provides a modest improvement of FEV1 but no improvement in mortality. ## Footnote It is rarely used in clinical practice.
56
What is the most effective method for smoking cessation?
Smoking cessation has the biggest effect on reducing mortality. ## Footnote Nicotine dependence is sensitive to the symptom of time to first cigarette after waking.
57
What is the efficacy of Nicotine Replacement Therapy (NRT) in smoking cessation?
NRT with patch (27% quit), gum (24%), or combined (36%) are relatively safe and effective. ## Footnote Combined NRT has the highest quit rate.
58
What is Varenicline and its role in smoking cessation?
Varenicline is a nicotine receptor partial agonist and competitive antagonist, producing mild positive agonist effects on nicotine receptors. ## Footnote It blocks nicotine from binding receptors, reducing pleasurable sensations when smoking.
59
What are the indications for oxygen therapy in COPD?
Oxygen is indicated with PaO2 < 55 mm Hg, baseline oxygen saturation < 88%, or exertional desaturation on 6MWT. ## Footnote It improves mortality when used for >15 hours/day.
60
What is the purpose of pulmonary rehabilitation in COPD?
Pulmonary rehabilitation provides education, support, inhaler technique, and exercise regimes. ## Footnote It has proven benefits in symptoms, exacerbations, quality of life, and 6MWT.
61
What are the indications for lung volume reduction surgery?
Indicated with FEV1 20-45%, DLCO > 20%, and large upper lobe bullae. ## Footnote It is a palliative procedure to allow for better lung expansion.
62
What are the contraindications for BiPAP in COPD management?
Contraindications include reduced GCS < 9, haemodynamic instability, craniofacial injury, or pneumothorax. ## Footnote BiPAP is used for hypercapnic respiratory failure.
63
What is Light's Criteria used for?
Light's Criteria are used to assess whether a pleural effusion is exudative or transudative. ## Footnote An exudative effusion is present with any one of three specific parameters.
64
What is the most common cause of a transudative pleural effusion?
Heart failure is the most common cause of a transudative effusion. ## Footnote Other causes include liver failure and renal failure.
65
What is the initial management for small to moderate pleural effusions?
Most can be managed conservatively with observation. ## Footnote Drainage can be considered for symptomatic relief.
66
What defines a tension pneumothorax?
Tension pneumothorax presents with haemodynamic instability, dyspnoea, chest pain, and tracheal deviation. ## Footnote An X-Ray should not be performed prior to intervention.
67
What is the typical presentation of mesothelioma?
Typical presenting complaints include chest wall pain or dyspnoea from a malignant pleural effusion. ## Footnote Mesothelioma is an aggressive pleural-based malignancy linked to asbestos exposure.
68
What is the leading cause of cancer-related death in Australia?
Lung cancer is the leading cause of cancer-related death in Australia. ## Footnote The majority are non-small cell lung cancer (85%).
69
What is the significance of driver mutations in lung cancer treatment?
Driver mutations, such as EGFR mutations in metastatic adenocarcinoma, are crucial for prognosis and treatment. ## Footnote Staging and prognosis depend on histological classification and stage of disease.
70
What is essential for the diagnosis of non-small cell lung cancer?
Histological confirmation of non-small cell lung cancer is essential.
71
Which subtype of non-small cell lung cancer has overtaken squamous cell carcinoma?
Adenocarcinoma has overtaken squamous cell carcinoma as the most common subtype.
72
What is crucial for adenocarcinoma in terms of treatment?
Cytological evaluation for driver mutations such as EGFR mutants is essential.
73
What imaging technique has revolutionized lung cancer staging?
PET-CT imaging has revolutionized lung cancer staging.
74
What percentage of new presentations does PET-CT up-stage compared to standard CT?
PET-CT up-stages disease in over 50% of new presentations.
75
What cannot be used for screening in lung cancer due to limitations?
PET-CT cannot be used for screening, as it may not detect nodules <10mm.
76
What classification system is used for staging non-small cell lung cancer?
The TNM classification system is used for staging NSCLC.
77
What characterizes Stage I lung cancer?
Stage I = Solitary tumour; 1A <3cm, 1B >3cm.
78
What is the standard treatment for Stage I non-small cell lung cancer?
Surgical lobectomy is generally curative for Stage I disease.
79
What is the standard of care for Stage II non-small cell lung cancer?
Adjuvant immuno/chemotherapy with cisplatin-based chemotherapy for 4 cycles.
80
What are the treatment options for Stage III non-small cell lung cancer?
Consideration of surgical resection or combined platinum-based immuno/chemo/radiotherapy.
81
What is the prognosis for Stage IV lung cancer?
Stage IV disease is incurable with palliative intent treatments.
82
What is the 5-year survival rate for Stage IV lung cancer with standard chemotherapy?
Approximately 5% with a median survival of approximately 12 months.
83
What types of inhibitors are used in the treatment of non-small cell lung cancer?
PD-1 Inhibitors and CTLA-4 Inhibitors.
84
What should be measured on malignant cells for immunotherapy response?
PD-L1 expression should be measured on malignant cells.
85
What is the typical patient profile for acquired EGFR mutations in metastatic lung adenocarcinoma?
Young, female, Asian, non-smoker.
86
What treatment is emerging for ALK gene mutations?
Drugs such as Crizotinib.
87
What is a common complication of non-small cell lung cancer?
Malignant pleural effusions are common.
88
How are malignant pleural effusions typically managed?
Managed with intercostal catheters for drainage and talc pleurodesis.
89
What can occur as a complication of malignant pericardial effusions?
Haemodynamic cardiac tamponade may require pericardiocentesis.
90
What sign indicates superior vena cava obstruction?
Positive Pemberton's Sign.
91
What is the median survival for limited stage small cell lung cancer?
Median 12 months survival with chemotherapy.
92
What is the treatment for limited stage small cell lung cancer?
Combined chemoradiotherapy with Cisplatin plus Etoposide.
93
What is the prognosis for extensive stage small cell lung cancer?
Median survival of <9 months.
94
What paraneoplastic syndrome is associated with small cell lung cancer?
Syndrome of inappropriate ADH secretion resulting in hyponatraemia.
95
True or False: Small cell lung cancer is more common than non-small cell lung cancer.
False.
96
What is Virchow's Triad?
A summary of the predisposition to venous thromboembolism, consisting of: * Hypercoagulable state * Venous stasis * Endothelial damage ## Footnote Hypercoagulable states include hereditary factors like Factor V Leiden and acquired factors like cancer and pregnancy.
97
What are some hereditary causes of a hypercoagulable state?
Hereditary causes include: * Factor V Leiden * Protein C deficiency * Protein S deficiency * Antithrombin deficiency ## Footnote These genetic factors increase the risk of thrombus formation.
98
What are some acquired causes of a hypercoagulable state?
Acquired causes include: * Cancer * Dehydration * Pregnancy * Polycythaemia * Antiphospholipid Syndrome ## Footnote These conditions can lead to increased thrombus formation.
99
What does a low Well's score indicate?
A low Well's score (less than 2) indicates low risk for venous thromboembolism (VTE). ## Footnote This score is used for risk stratification in patients.
100
What is the purpose of the D-Dimer assay?
The D-Dimer assay detects fibrin breakdown products to help diagnose VTE. ## Footnote A normal D-Dimer can help rule out VTE in low-risk patients.
101
What is considered the gold standard test for VTE in the Emergency Department?
CT Pulmonary Angiography (CTPA) ## Footnote It uses intravenous iodine contrast media to visualize the pulmonary vasculature.
102
What does a positive CTPA indicate?
A positive CTPA indicates contrast filling defects in the pulmonary vasculature. ## Footnote It is highly sensitive for detecting central, large calibre arterial thrombi.
103
What is the main finding in an ECG for acute pulmonary embolism?
Sinus tachycardia is the most common finding. ## Footnote Other signs may include right bundle branch block and ST segment changes.
104
What are the echocardiographic signs of acute pulmonary embolism?
Signs include: * Increased right ventricular size * Decreased right ventricular systolic function * Tricuspid regurgitation * McConnell's sign ## Footnote McConnell's sign is specific for acute PE.
105
What is the initial management for a patient with systolic hypotension and confirmed central pulmonary artery thromboembolism?
Thrombolysis should be considered unless contraindicated. ## Footnote This is crucial for restoring hemodynamic stability.
106
What should be used to prevent further thrombus formation after initial treatment?
Unfractionated heparin should be used subsequently. ## Footnote This is part of the management strategy for pulmonary embolism.
107
What is the recommended duration of treatment for acute pulmonary embolism?
Treatment should be continued for at least 3 months. ## Footnote Unprovoked PE typically requires at least 6 months of treatment.
108
What is the average rate of recurrence for VTE within 12 months?
The average rate of recurrence is 10% within 12 months. ## Footnote Risk factors for recurrence include active cancer and unprovoked VTE.
109
What is the WHO classification of pulmonary hypertension (PHTN)?
PHTN is split into five main groups: * Group 1 - Pulmonary Arterial Hypertension * Group 2 - Left heart disease * Group 3 - Lung disease * Group 4 - Chronic thromboembolic pulmonary hypertension (CTEPH) * Group 5 - Others/Mixed ## Footnote Each group has distinct causes and treatment approaches.
110
What is the typical index symptom of pulmonary hypertension?
The typical index symptom is exertional dyspnoea. ## Footnote Diagnosis often takes multiple visits to healthcare providers.
111
What is the Six Minute Walk Test (6MWT) used for?
It is used to assess functional capacity and oxygen desaturation during exercise. ## Footnote It serves as a primary endpoint in clinical trials for pulmonary hypertension treatments.
112
What is a Right Heart Catheter (RHC) used to assess?
RHC is used to assess the aetiology and severity of pulmonary arterial hypertension. ## Footnote It is more sensitive than echocardiography for determining severity.
113
What is the procedure for RHC?
Passing a Swan-Ganz catheter percutaneously from the internal jugular vein or femoral vein ## Footnote The catheter is progressed through the right atrium, right ventricle, and into the pulmonary artery.
114
What pressures are measured during RHC?
Pressure measurements are made at: * Right atrium * Right ventricle * Pulmonary artery ## Footnote A wedge pressure is taken by inflating a balloon within the pulmonary artery.
115
What indicates pulmonary hypertension (PHTN) based on mean pulmonary artery pressure (mPAP)?
A mean pulmonary artery pressure (mPAP) of 25mmHg or greater is diagnostic of PHTN.
116
How is cardiac output (CO) measured?
Cardiac output (CO) is measured via thermodilution.
117
What does a pulmonary wedge pressure of 15mmHg indicate?
Indicates PHTN due to left heart disease.
118
What is a vasoreactivity test used for?
A positive test indicates the need for calcium channel blockers in NYHA class II-IV.
119
What does increased pulmonary vascular resistance (PVR) indicate?
An increased PVR is pathognomic of pulmonary hypertension.
120
What is the formula for calculating Pulmonary Vascular Resistance (PVR)?
PVR = (mPAP - PWP) + CO.
121
What is the importance of CT Pulmonary Angiography in PHTN?
It is used to exclude chronic thromboembolic pulmonary arterial hypertension (CTEPH).
122
What are typical findings in CTEPH?
* Proximal chronic thrombi with recanalization of flow * Mosaic attenuation of distal flow * Decreased peripheral sub-segmental blood flow ## Footnote Iodine mapping can further support the diagnosis of CTEPH.
123
What does a V/Q scan indicate in CTEPH?
Extensive, multilobar V/Q mismatching with reduced perfusion.
124
What is the role of pulmonary angiography in CTEPH diagnosis?
Considered if CTPA and V/Q images are suggestive of CTEPH.
125
What are the major pathways for Group 1 treatment of PHTN?
* Endothelin receptor antagonists * Prostacyclin * Nitric oxide ## Footnote Major clinical trials have occurred in this area.
126
What is the action of endothelin in the body?
Acts on the endothelin receptor on smooth muscle cells, causing proliferation and vasoconstriction.
127
What is the significance of macitentan?
Has data on improved combined morbidity and mortality.
128
What are common side effects of Ambrisentan and Bosentan?
* Hepatotoxicity * Hypotension.
129
What is the mechanism of action of prostacyclin?
Promotes vasodilation and has anti-proliferative effects.
130
What is the only drug to demonstrate improved mortality in PHTN?
Prostacyclin.
131
What is the role of Riociguat in CTEPH treatment?
Improves 6MWT by 39m and modestly reduces PVR.
132
What is the primary treatment for Class II PHTN?
Managing left heart disease.
133
What is a pulmonary endarterectomy?
A surgical procedure that provides strong mortality benefit in suitable patients.
134
What complications are associated with obstructive sleep apnoea (OSA)?
* Cardiovascular disease * Hypertension * Polycythaemia * Aortic aneurysmal disease * Cerebrovascular accident * Pulmonary hypertension * Ischaemic heart disease * Atrial fibrillation * Heart failure. ## Footnote Particularly cor pulmonale from elevated pulmonary artery pressures.
135
What are classical symptoms of OSA?
* Daytime somnolence * Restless legs at night * Early morning headache on waking * Unrefreshing sleep.
136
What is the Epworth Sleepiness Score?
A validated screening tool for sleep apnoea involving 8 questions rated 0-3.
137
What does a normal Apnoea Hypopnea Index (AHI) indicate?
Normal <5, Mild 5–20, Moderate 20–40, Severe >40.
138
What is the significance of a repeated sleep latency of 0-5 minutes?
Strongly suggestive of narcolepsy.
139
What should be avoided in the management of OSA?
Avoidance of alcohol, depressants, and sedatives.
140
What is continuous positive airway pressure (CPAP)?
A device that delivers positive pressure to splint open the airways throughout the respiratory cycle.
141
What is the treatment implication for patients with a moderate or severe AHI?
Treatment of OSA should be reserved for these patients.
142
What is the impact of OSA on public health?
Increases motor vehicle accidents with an odds ratio of 6.3 compared to the control population.
143
What is the AHI threshold for treating OSA?
Moderate or severe AHI ## Footnote AHI stands for Apnea-Hypopnea Index, which measures the severity of sleep apnea.
144
What is a significant benefit of treating OSA?
Reduces motor vehicle accidents ## Footnote Treatment of OSA can significantly improve safety for drivers.
145
How does OSA treatment impact quality of life?
Improves quality of life and daytime sleepiness ## Footnote Patients often experience better overall well-being after treatment.
146
What is the average reduction in blood pressure from OSA treatment?
-3 mmHg ## Footnote This reduction is much less than that achieved with antihypertensive drug agents.
147
True or False: Treatment of OSA reduces the risk of cardiovascular disease.
False ## Footnote There is no evidence that treatment of OSA reduces the risk of cardiovascular diseases.
148
What is the goal of titrating devices for OSA treatment?
To reduce the AHI to normal ## Footnote This involves delivering enough pressure to avoid apneas.
149
What does CPAP stand for?
Continuous Positive Airway Pressure ## Footnote CPAP provides a continuous low level of pressure regardless of the respiratory cycle.
150
What is the difference between BiPAP and CPAP?
BiPAP provides higher inspiration pressure and lower expiration pressure ## Footnote This allows for normal breathing rates and senses airflow changes.
151
What is a characteristic feature of Central Sleep Apnoea (CSA)?
Modification of the central hypercapnic threshold ## Footnote This reduces the central waking stimulus.
152
What is a common cause of CSA?
Chronic hypercapnic respiratory failure ## Footnote Other causes include advanced heart failure or neuromuscular diseases.
153
What is narcolepsy?
Chronic neurological disorder affecting sleep-wake cycle ## Footnote Patients experience uncontrollable daytime sleepiness and may fall into REM sleep abruptly.
154
What percentage of narcolepsy patients experience cataplexy?
Approximately 70% ## Footnote Cataplexy can be triggered by strong emotions.
155
What is the gold standard for diagnosing narcolepsy?
Multiple Sleep Latency Test ## Footnote This test shows repeated rapid onset of REM sleep.
156
What are common treatments for narcolepsy?
Stimulants and Modafinil ## Footnote Side effects may include severe skin reactions and anxiety.
157
What is Restless Legs Syndrome (RLS)?
Strong urge to move legs, usually at night ## Footnote Symptoms worsen when relaxing or lying still.
158
What deficiency is strongly linked to RLS?
Iron deficiency ## Footnote Symptoms can improve with iron supplementation if deficient.
159
What is Periodic Limb Movement Disorder?
Involuntary leg movements during sleep ## Footnote These movements can cause unrefreshing sleep.
160
What is bronchiectasis?
Obstructive lung disease characterized by airway dilation ## Footnote It often results from chronic infections and inflammation.
161
What are common pathogens associated with bronchiectasis?
* Pseudomonas * Staphylococcus aureus * Non-Tuberculous Mycobacteria * Burkholderia ## Footnote These pathogens can colonize scarred airways.
162
What is the gold standard for diagnosing bronchiectasis?
Bronchial dilation greater than adjacent blood vessels on CT ## Footnote Other CT features may include signet rings and tree-in-bud appearance.
163
What is cystic fibrosis?
Multisystem genetic disorder with chronic complications ## Footnote It is caused by mutations in the CFTR gene.
164
What is the most common mutation in cystic fibrosis?
AF508 ## Footnote This mutation accounts for 66% of detected mutations.
165
What is the gold standard for diagnosing cystic fibrosis?
Genetic testing demonstrating known disease-causing mutation ## Footnote Sweat chloride testing can also confirm the diagnosis.
166
What are common respiratory complications in cystic fibrosis?
* Sinusitis * Bronchiectasis * Pseudomonas aeruginosa colonization ## Footnote These complications often lead to hospital admissions.
167
What is the role of Ivacaftor in cystic fibrosis treatment?
Potentiates CFTR by maintaining the channel open ## Footnote It is specifically for the G551D mutation and improves FEV1.
168
What is a key aspect of cystic fibrosis management?
Secretion management and anti-infectives ## Footnote Treatment aims to manage multi-system complications.
169
What are the effects of hepatotoxicity in relation to drug interactions?
Oral contraceptive pill drug interaction through CYP3A4 ## Footnote CYP3A4 is a member of the cytochrome P450 family involved in drug metabolism.
170
What is the indication for lung transplantation?
FEV1 < 30% predicted, PaO2 < 55 mmHg, PaCO2 > 50 mmHg ## Footnote These criteria indicate severe lung dysfunction requiring transplantation.
171
What is the survival benefit of lung transplantation?
Transplant offers a 5-year survival of approximately 65% ## Footnote Survival rates can vary based on individual patient conditions and post-transplant care.
172
What is the purpose of pancreatic enzyme supplementation?
To overcome exocrine pancreas insufficiency ## Footnote This is important for patients with pancreatic dysfunction to aid digestion.
173
What monitoring is required for liver health in patients with concerns over cirrhosis?
Regular liver function test monitoring and abdominal ultrasounds ## Footnote These tests help in early detection of liver complications.
174
Which medication has evidence in biliary disease?
Ursodeoxycholic acid ## Footnote It is used to treat certain liver and gallbladder conditions.
175
What therapy may be required for Gastroesophageal reflux disease?
Proton pump inhibitor therapy ## Footnote These drugs reduce stomach acid production to alleviate symptoms.
176
What supplementation is required to address fat-soluble vitamin deficiencies?
Multivitamin supplementation, specifically ADEK ## Footnote Vitamins A, D, E, and K are crucial for various bodily functions.
177
What is recommended for bone health in patients?
Vitamin D supplementation with cholecalciferol ## Footnote Cholecalciferol is a form of vitamin D important for calcium absorption.
178
What treatment may be indicated for patients with chronic sinusitis?
Nasal steroids sprays ## Footnote These sprays help reduce inflammation in the nasal passages.
179
What is often required for male infertility?
Assisted reproductive therapy ## Footnote This includes techniques such as IVF and ICSI.
180
What is the most successful method of fertility treatment using sperm injections?
Intracytoplasmic sperm injections (ICSI) ## Footnote ICSI involves injecting a single sperm directly into an egg.
181
What characterizes Bronchiolitis Obliterans?
Peribronchial fibrosis with extrinsic compression of the airway ## Footnote This condition can lead to significant airway obstruction.
182
What imaging shows the characteristic findings of Usual Interstitial Pneumonia (UIP)?
HRCT shows reticular opacities and honeycombing ## Footnote These findings are indicative of progressive lung fibrosis.
183
What is the prognosis for patients with Usual Interstitial Pneumonia?
Median survival of 3-5 years ## Footnote Prognosis is generally poor due to the progressive nature of the disease.
184
What antifibrotic therapies are effective in treating Usual Interstitial Pneumonia?
Pirfenidone and Nintedanib ## Footnote Both medications have shown to reduce disease progression.
185
What is the typical imaging finding for Non-specific interstitial pneumonia?
Basal, sub pleural ground glass changes but no honeycombing ## Footnote These findings can help differentiate it from other forms of lung disease.
186
What is the common treatment for Cryptogenic Organising Pneumonia?
Steroids and treatment of the underlying disease ## Footnote Steroids can help reduce inflammation and improve symptoms.
187
What is the primary cause of Desquamative Interstitial Pneumonia?
Smoking-related parenchymal lung disease ## Footnote Smoking cessation is crucial for treatment and recovery.
188
What imaging findings are associated with Silicosis?
Upper to mid zone interstitial fibrosis and 'egg shell' lymphadenopathy ## Footnote These findings are characteristic of silicosis due to silica exposure.
189
What is the role of corticosteroids in the treatment of Sarcoidosis?
Corticosteroids are the treatment of choice for stage 3-4 disease ## Footnote They help manage inflammation associated with the disease.
190
What is Lofgren syndrome?
Hilar lymphadenopathy, erythema nodosum, arthralgia ## Footnote It is a specific presentation of sarcoidosis.
191
What is the typical presentation timeline for hypersensitivity pneumonitis?
Flu-like symptoms 4-8 hours post exposure ## Footnote Symptoms resolve within 24-48 hours but recur with further exposure.