week 9 Flashcards

(88 cards)

1
Q

Restrictive lung disorders

A

group of pulmonary conditions that
restrict the expansion of lungs leads to a decreased total lung volume

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2
Q

Intrinsic conditions (restrictive lung disorders)

A
  • Umbrella term for diseases that affect lung parenchyma as opposed to airways through inflammation and fibrosis
    eg interstitial lung diseases
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3
Q

extrinsic restrictive lung disorders

A

chest wall deformities (e.g., scoliosis, kyphosis, pectus abnormalities), pleural diseases

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4
Q

Intersititium and Pleura disorders

A

These disorders are characterised by a reduced distensibility of the lungs, compromising lung expansion, and, in turn, reduced lung volumes, particularly with reduced total lung capacity

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5
Q

Restrictive lung disorders can be caused by

A

Pulmonary parenchyma diseases (intrinsic causes) involve the lung parenchyma itself
Extrapulmonary diseases (extrinsic causes) originate from neuromuscular disorders, obesity and other extra-parenchymal disorder

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6
Q

Aetiology
Pulmonary Parenchyma Diseases (Intrinsic Causes)

A

Idiopathic pulmonary fibrosis (IPF)
Non-specific interstitial pneumonia (NSIP)
Cryptogenic organizing pneumonia (COP)
Sarcoidosis
Acute interstitial pneumonia (AIP)

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7
Q

Aetiology Extrinsic or Extrapulmonary Diseases

A

Kyphoscoliosis
Pleural conditions such as effusions, trapped lung, pleural scarring, chronic empyema, asbestosis
Obesity
Neuromuscular disorders like muscular dystrophy, amyotrophic lateral sclerosis, polio, and phrenic neuropathies

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8
Q

Interstitial lung disease Aetiology

A

Occupational and environmental
* Asbestosis, silicosis, inhalation of toxins
Treatment related/drug induced:
* Bleomycin, radiation induced lung injury
Connective tissue disorders:
* RA, ankylosing spondylitis, SLE
Idiopathic
* Sarcoidosis, idiopathic interstitial
pneumonia, familial

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9
Q

Interstitial lung disease CM

A
  • dyspnoea
  • progressive breathlessness with exertion
  • exertional intolerance
  • persistent nonproductive cough
  • haemoptysis
  • abnormal chest imaging
  • lung function abnormalities
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10
Q

Occupational lung diseases and predisposing factors

A

Inhalation of dust, fumes, smoke, biological agents
Predisposing factors: pre-existing lung condition, exposure, duration, concentration, size of particles affect onset and

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11
Q

Pneumoconiosis
Investigations
CXR

A
  • varies with disease severity
  • micronodular mottling and haziness
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12
Q

Pneumoconiosis
Investigations
HRCT

A
  • pattern of parenchymal involvement
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13
Q

Pneumoconiosis – PFTs and ABGs
lung volumes

A

Restrictive defect with reductions in:
* Total Lung Capacity (TLC)
* Functional Residual Capacity (FRC)
* Residual volume (RV)

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14
Q

Pneumoconiosis – PFTs and ABGs
Spirometry

A

decreased:
* Forced vital capacity (FVC)
* Forced expiratory volume in one second
(FEV1)
* changes in proportion
* FEV1/FVC ratio normal or increased (80%)

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15
Q

Pneumoconiosis – PFTs and ABGs
Multiple physiologic derangements
ABG

A

Multiple physiologic derangements
* diffusion limitation
* ventilation–perfusion mismatching
* abnormalities of pulmonary vasculature
Resulting in hypoxaemia

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16
Q

Pneumoconiosis – PFTs and ABGs

A
  • DLCO decreased
  • diffuse alveolar capillary damage
  • loss of aerated alveoli
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17
Q

Asbestosis caused by

A

asbestos fibre inhalation

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18
Q

Asbestos

A

Naturally occurring fibres composed of hydrated magnesium silicates

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19
Q

Asbestosis aeitology

A

Direct work-related environmental exposure; Bystander exposure

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20
Q

pleural space

A
  • Parietal – lines chest cavity
  • Visceral – lines lungs
  • Potential space: 5–15 ml of fluid
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21
Q

Pleural cavity: negative pressure

A

-8 cmH2O during inspiration
-4 cmH2O during expiration
Lubricating fluid of the pleural surface
No more than 15 ml of serous, relatively acellular and clear fluid

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22
Q

Pneumothorax is the air in the

A

Air in the pleural space (between the visceral and parietal pleura

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22
Q

Pneumothorax disorders are

A

Disruption to the negative pressure in IPS, rupture of subpleural cyst
Visceral pleura separates from parietal, air enters IPS, air pushes/collapses the lung down

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23
Q

Pneumothorax Aetiology

A

spontaneous; pre-existing pulmonary disease; tension pneumothorax

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24
Pneumothorax CM
normal to diaphoretic and unwell
25
Pneumothorax CXR
* separation between lungs and pleura
26
Pneumothorax ABG
* decreased PaO2 * acute respiratory alkalosis if high RR
27
Pneumothorax PFT
* not routinely taken
28
Pleural Effusion
Pathologic collection of fluid or pus in pleural cavity
29
Pleural Effusion Aetiology
: (1) transudates, (2) exudates, (3) empyema attributable to infection in the pleural space, (4) haemothorax or haemorrhagic pleural effusions, (5) chylothorax or lymphatic pleural effusions
30
Pleural Effusion Pathophysiology
Pleural fluid rate exceeds lymphatic removal rate in the pleural cavity
31
Pleural Effusion Pathogenesis
Transudates - systemic factors that alter the pressure and fluid balance in the body. Exudates - caused by inflammation or injury to the pleura itself
32
Pleural Effusion Clinical Manifestations
Dyspnpea, pleuritic pain that is sharp and worsens with inspiration, dry cough, decreased chest wall movement, absence of breath sounds
33
Pleural Effusion CXR
* >200 ml of fluid - costophrenic angle * meniscus sign
34
Pleural Effusion PFTs
* not usually assessed * restrictive lung disorder
35
Pleural Effusion ABGs
* can worsen gas exchange * hypoxaemia
36
Haemothorax
collection of blood in the space between visceral and parietal pleura
37
Haemothorax. Aetiology
traumatic injury; aortic rupture, myocardial rupture, injuries to hilar structures; injuries to lung parenchyma and intercostal or mammary blood vessels; disruption of intercostal vessels, especially with # multiple ribs
38
Haemothorax CM
respiratory distress and tachypnoea
39
Haemothorax Pathophysiology
bleeding into hemithorax from injuries; severity of pathophysiologic response depends on the location of the injury, the patient's functional reserve, the volume of blood, and the rate of accumulation in the hemithorax. Blood in the pleural space affects the functional vital capacity of the lung by creating alveolar hypoventilation, V/Q mismatch, and anatomic shunting
40
Scoliosis
Lateral displacement/curvature of spine in coronal plane
41
Scoliosis Aetiology
* idiopathic (no definite aetiology) * de novo scoliosis * congenital vertebral anomalies * connective tissue disorders, inflammatory conditions, NMD, Marfan syndrome
42
Scoliosis CM
* dyspnoea on exertion * rapid, shallow breathing * thoracic cage deformity
43
Scoliosis Investigations
PFTS: restrictive pattern * ▼ TLC and VC * preserved RV; RV/TLC ratio ▲
44
Scoliosis ABGs
alveolar hypoventilation * hypercapnia and hypoxaemia
45
Kyphosis is
A/P angulation of spine Severe rib distortion when: * angulation of kyphosis is excessive * moderate to severe scoliosis
46
Kyphosis Aetiology
trauma, developmental problems or degenerative diseases
47
Kyphosis Clinical manifestations
* dyspnoea on exertion * rapid, shallow breathing * chest wall deformity
48
Kyphosis Investigations
* restrictive ventilation impairment (Lorbergs et al, 2023) *  kyphosis (Cobb angle)  FVC (Ailon et al, 2015) *  FEV1
49
Kyphosis ABGs
* hypoxaemia: ventilation perfusion mismatch * alveolar hypoventilation
50
Scoliosis Pathogenesis
* paediatric-adolescent-adulthood development * compression of growth plates * asymmetric loading * higher loads on chondrocytes (concave side)
51
Kyphosis Pathogenesis
* Vertebral compression fractures * Low bone density * Scheuermann's disease * Degenerative disc disease * Postural changes
52
Ankylosing Spondylitis
chronic inflammation at the site of ligamentous insertion into the spine or sacroiliac joints
53
Ankylosing Spondylitis Aetiology
* genetic and environmental factors * HLA-B27 Gene * hereditary component
54
Ankylosing Spondylitis CM
* lower back pain * stiffness after prolonged rest, decrease with exercise * limited flexibility of back and neck * Associated: arthritis, uveitis, spondylitic heart disease, pulmonary fibrosis, and polyarteritis
55
Ankylosing Spondylitis Investigations
PFTs * restrictive lung dysfunction (thorax, ILD) * bronchiectasis develops – obstructive also
56
Ankylosing Spondylitis Pathogenesis
* inflammatory process (tumour necrosis factor - crucial role) affects articular processes, costovertebral joints, sacroiliac joints * induce a fibrotic response * leads to joint calcification, ligament ossification, and skeletal immobility
57
Ankylosing Spondylitis Pathophysiology
* starts with inflammation at the entheses * over time chronic inflammation causes bone erosion and new bone formation * can lead to fusion of vertebrae
58
Pectus excavatum
concave depression; broad shallow defect or narrow central pocket
59
Pectus excavatum CM
* mild dyspnoea on exertion during exercise * pain in rib deformity area
60
Pectus excavatum: Investigations
normal – restrictive PFTs severity dependent
61
Pectus carinatum
protrusion of the sternum and costal cartilages
62
Pectus carinatum Clinical manifestations
* exertional dyspnoea * frequent respiratory infections
63
PFTs Pectus carinatum Investigations
normal PFTs
64
Obesity
* elevated body mass index (BMI) - BMI > 30 kg/m2 * class 1 (BMI of 30 to <35), class 2 (BMI of 35 to <40), and class 3 (BMI of >40)
65
Pathogenesis Obesity
* sustained positive energy balance * resetting of the body weight “set point” at an increased value * habitual consumption of highly palatable and energy-dense diets predispose to excess weight gain irrespective of macronutrient content. * beyond diet, environmental factors confer obesity risk.
66
obesity respiratory investigations ABGs
Hypoventilation: hypoxemia and hypercapnia
67
obesity respiratory investigations PFTs
* Static lung volumes:  chest wall compliance, VC, TLC, and expiratory reserve * Spirometry: Obstruction (e.g. asthma – BMI) * Airway hyperresponsiveness (e.g.leptin)
68
Neuromuscular diseases
Diseases affecting the muscles of respiration or their nerve supply can lead to dyspnoea and respiratory failure
69
Pulmonary vascular disease (PVD)
includes a number of conditions that affect the blood vessels in the lungs of which the most common are pulmonary hypertension and pulmonary emboli.
70
Pulmonary hypertension and mPAP
refers to elevated pressure in the pulmonary arteries a mean pulmonary artery pressure (mPAP) >20 mmHg at rest
71
Pulmonary Hypertension Aetiology
Pulmonary arterial hypertension (PAH) left heart disease Chronic lung disease and/or hypoxia Chronic thromboembolic pulmonary hypertension (CTEPH) Multifactorial/unclear mechanisms
72
Pulmonary Hypertension risk factors
Family history (heritable PAH) Connective tissue diseases (e.g., systemic sclerosis Congenital heart disease History of thromboembolism HIV infection
73
Pathophysiology of pulmonary hypertension
1. Initial Trigger: Vascular Injury or Stress 2. Abnormal Changes in the Pulmonary Arteries 3. Increased Pulmonary Vascular Resistance (PVR) 4. Right Ventricular (RV) Strain and Failure
74
CTPEH pathogenesis
organised thrombi become incorporated into the vessel wall, leading to fibrosis, intimal thickening, and luminal narrowing or obliteration This persistent obstruction increases PVR and pulmonary artery pressures Plus there is secondary microvascular remodelling
75
CTPEH CM early symptoms
Exertional dyspnoea Fatigue Lethargy
76
CTPEH CM late symptoms
Exertional chest pain Exertional syncope Peripheral oedema Right upper quadrant abdominal pain and swelling Weight gain
77
CTPEH signs
Cardiac: e.g., loud, palpable second heart sound Elevated jugular venous pressure (JVP) Hepatomegaly (pulsatile, tender liver) Peripheral oedema Ascites Pleural effusions
78
Pulmonary Embolism
refers to obstruction of the pulmonary artery or one or more of its branches by material that originated elsewhere in the body
79
Pulmonary Embolism Aetiology
Thromboembolism Fat embolism Amniotic fluid embolism Tumour embolism Air embolism Foreign body embolism
80
Risk factors for thrombotic PE include
Surgery Trauma Older age (≥65 years) Immobilisation/limb immobility Cancer
81
Risk factors for non-thrombotic emboli include
Fractures (fat embolism) Pregnancy and obstetric procedures (amniotic fluid embolism) Trauma, surgery, or central venous catheterisation (air embolism) Malignancy (tumour embolism - rare, end-stage manifestations of malignancy)
82
Thromboembolic PE Pathogenesis
1. Thromboembolic PE- Mechanical obstruction of pulmonary arteries by a clot leads to impaired blood flow
83
Pulmonary Embolism CM
Acute dyspnoea (most common) Pleuritic chest pain Tachypnoea Tachycardia Hypoxaemia
84
Fat Embolism Syndrome Pathogenesis
Fat globules enter venous circulation from injured marrow obstructing the capillaries
85
Air Embolism PE pathogenesis
Air enters the venous system and reaches the pulmonary circulation
86
Amniotic Fluid Embolism PE pathogenesis
Entry of amniotic fluid and foetal debris into maternal circulation.
87
Tumour Embolism PE pathogenesis
Tumour cells enter pulmonary arteries and obstruct microvasculature