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Kendigs Respiratory Diseases > Chest Wall > Flashcards

Chest Wall Flashcards

1
Q

What is the principal muscle used for inspiration in normal resting conditions?

A

Diaphragm

The diaphragm is the primary muscle responsible for drawing air into the lungs during normal, quiet breathing.

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2
Q

What happens to expiratory muscles during increased demands for air pumping?

A

They become activated during the 2nd phase of expiration

This activation helps decrease the end expiratory volume below functional residual capacity (FRC).

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3
Q

What is a significant effect of sleep on lung mechanics?

A

Leads to a decrease in FRC

This is primarily due to the cephalad movement of abdominal contents.

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4
Q

What phenomenon occurs in infants due to compliant rib cages during inspiration?

A

Paradoxical inward rib cage movement

This occurs due to decreased tonic activity of intercostal and upper airway muscles.

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5
Q

What is often the first sign of chronic respiratory failure in progressive neuromuscular disorders?

A

Nocturnal hypoventilation with alteration of blood gases

Conditions like Duchenne muscular dystrophy exhibit this symptom.

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6
Q

What are the three features of the rib cage that predispose newborns to respiratory failure?

A
  • Very compliant rib cage due to nonossification of the ribs
  • Intercostal muscles unable to add volume with the ‘bucket handle’ motion
  • Perpendicular insertion of the diaphragm to the rib cage

These factors contribute to diaphragmatic fatigue and inadequate ventilation.

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7
Q

What is the compliance of the chest wall at birth compared to lung compliance?

A

Chest wall compliance is up to 7-fold greater than lung compliance at birth

This compliance reduces by half by the age of 3.

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8
Q

What compensatory mechanism do newborns use to maintain functional residual capacity?

A

Increasing respiratory rate

This reduces the time devoted to lung deflation.

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9
Q

What are the causes of respiratory muscle fatigue in newborns?

A
  • Nutritional difficulties
  • Hypoglycemia
  • Hypocalcemia
  • Hypophosphatemia
  • Acidosis

These factors amplify the risk of respiratory muscle fatigue.

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10
Q

What is the primary cause of respiratory failure in the newborn?

A

Respiratory muscle fatigue

This is more common in newborns than at any other time in life.

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11
Q

What is the impact of hyperinflation due to intrathoracic airway obstruction on the diaphragm?

A

Flattens the diaphragm contour and reduces inspiratory force

This occurs as lung volume increases above FRC.

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12
Q

What are some disorders that can lead to diaphragm dysfunction?

A
  • Neuropathic processes (e.g., quadriplegia)
  • Myopathic processes (e.g., muscular dystrophies)
  • Conditions producing primary respiratory disease
  • Blood flow reduction to the diaphragm
  • Hypoxemia at high altitude
  • Acute hypercapnia

These disorders can alter the functional properties of the diaphragm.

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13
Q

What is the consequence of certain therapies on respiratory muscles?

A

Lead to respiratory muscle dysfunction due to injury and atrophy

Examples include paralytics, corticosteroids, and controlled mechanical ventilation.

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14
Q

What mechanisms are activated to meet respiratory muscle work requirements during increased loads?

A
  • Increase in respiratory drive
  • Recruitment of diaphragm motor units
  • Increased recruitment of fast-twitch muscle fibers
  • Recruitment of accessory respiratory muscles
  • Change in respiratory patterns

These adaptations help cope with mechanical loads but can lead to fatigue.

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15
Q

What are the first symptoms of chronic respiratory muscle fatigue?

A
  • General fatigue
  • Dyspnea on exertion

Additional symptoms may include sleep hypoventilation and morning headaches.

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16
Q

What are the primary causes of chest wall movement disturbances?

A
  • Neurological diseases affecting CNS or peripheral innervation
  • Myopathies from muscular dystrophy
  • Disorders of the bony structures of the chest wall
  • Obesity

These conditions can lead to secondary dysfunction of the respiratory muscles.

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17
Q

What is diaphragm paralysis and its primary cause?

A

Results from injury to the phrenic nerve

This can occur during thoracic or neck surgery or due to other causes like tumors.

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18
Q

What is the clinical presentation of unilateral diaphragm paralysis?

A

Usually asymptomatic but can lead to exercise limitation

Symptoms may not be noticeable until physical exertion.

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19
Q

How is unilateral diaphragm paralysis diagnosed?

A
  • Chest x-ray
  • Fluoroscopy with a sniff test
  • Electromyography

These methods help confirm the presence of paralysis.

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20
Q

What is the treatment for symptomatic diaphragmatic paralysis?

A
  • Ventilatory support
  • Surgical plication if symptoms persist

The timing for surgery in infants is controversial.

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21
Q

What is the pathogenesis of spinal muscular atrophies?

A

Due to a deficient survival motor neuron (SMN) protein

This mutation leads to degeneration of anterior horn cells and skeletal muscle weakness.

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22
Q

What characterizes Spinal Muscular Atrophy Type I?

A
  • Poor muscle tone and weakness at 0–6 months
  • Inability to sit without support
  • Absence of sensory loss
  • Normal intellect

Prognosis is poor, with 95% of infants dying from respiratory failure by 18 months.

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23
Q

What are the diagnostic methods for spinal muscular atrophy?

A
  • Molecular genetic testing
  • Electromyography
  • Muscle biopsy

These tests help confirm the diagnosis and assess the extent of muscle involvement.

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24
Q

What therapeutic interventions are recommended for Spinal Muscular Atrophy Type I?

A
  • Palliative care
  • Proactive respiratory interventions

Focus on improving quality of life and managing respiratory symptoms.

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25
What does electromyography reveal in patients with denervation?
Diminished motor action potential amplitude ## Footnote Motor and sensory nerve conduction velocities are normal.
26
What is the focus of therapeutic interventions for Spinal Muscular Atrophy Type I?
Increasing the quality of life of the child ## Footnote Must be preceded by information to the family on treatment burden and prognosis.
27
List some components of a palliative care program for Spinal Muscular Atrophy Type I.
* Aspiration of secretions * Nasogastric feeding * Nasal oxygen * Morphine/sedation for dyspnea * Transient ventilation if needed
28
What kind of respiratory interventions are required for Spinal Muscular Atrophy Type II?
* Noninvasive ventilation * Mechanical in-exsufflation * Prevention of respiratory infections
29
What characterizes the respiratory involvement in Spinal Muscular Atrophy Type III?
Minimal respiratory involvement ## Footnote Does not require ventilatory support, or only in adult life.
30
What is Juvenile Myasthenia Gravis?
Acquired autoimmune disorder of neuromuscular transmission ## Footnote Associated with circulating autoantibodies against acetylcholine receptors in 80%-90% of cases.
31
What symptoms characterize Neonatal Myasthenia Gravis?
* Feeding and respiratory difficulties * Hypotonia * Weak cry * Facial weakness * Ptosis
32
What defines Congenital Myasthenic Syndromes?
Inherited disorders from mutations of genes crucial for neuromuscular transmission ## Footnote At least 24 different genes are known to cause these syndromes.
33
What is a common clinical presentation of Juvenile Myasthenia Gravis?
Fatigable muscle weakness that improves with rest ## Footnote Can include ocular symptoms like ptosis and diplopia.
34
How is Neonatal Myasthenia Gravis diagnosed?
Presence of myasthenia gravis in the mother ## Footnote Transient improvement after administration of edrophonium chloride or neostigmine.
35
What is the treatment for Juvenile Myasthenia Gravis?
* Anticholinesterase medication (pyridostigmine bromide) * Prednisone * Thymectomy * Respiratory support during acute exacerbations * IV immunoglobulins and plasmapheresis
36
What is Duchenne Muscular Dystrophy?
Most common and severe form of muscular dystrophy ## Footnote X-linked recessive trait with an incidence of approximately 1:3500 male births.
37
What is the role of dystrophin in muscle cells?
Links the normal contractile apparatus to the sarcolemma in skeletal muscle.
38
Describe the clinical presentation of Duchenne Muscular Dystrophy.
* Proximal muscle weakness at 2-4 years * Loss of ambulation by 10-12 years * Progressive respiratory problems * Restrictive syndrome due to muscle weakness
39
What are some common symptoms of Myotonic Dystrophy?
* Myotonia * Muscle weakness * Cataracts * Cardiac dysrhythmias * Sleep disorders
40
What is the diagnostic hallmark of Duchenne Muscular Dystrophy?
Elevated serum CK concentration (50-100 times the reference range) in a young boy.
41
What is Pectus Excavatum?
Symmetric or asymmetric depression of the sternum and anterior chest ## Footnote Occurs due to defects in the sternum cartilage or overgrowth of costal cartilage.
42
What is the most common form of scoliosis?
Idiopathic Kyphoscoliosis ## Footnote Accounts for 80%-85% of cases and is defined as a spine deformity without overt malformations.
43
What is the definition of thoracic insufficiency syndrome?
Conditions that impair lung function or postnatal lung growth due to spine and chest wall disorders.
44
What characterizes Asphyxiating Thoracic Dystrophy?
Narrow hypoplastic rib cage and generalized chondrodystrophy with short limb dwarfism.
45
What is the treatment approach for respiratory disability in muscular dystrophies?
Directed at slowing down progression of respiratory insufficiency while ensuring good quality of life.
46
What are some key management strategies for acute respiratory deteriorations?
* Avoid contact with respiratory infections * Preventive immunization * Early appropriate antibiotic treatment
47
What is the mechanism of action of corticosteroid therapy in Duchenne Muscular Dystrophy?
Anti-inflammatory effect that may delay the depletion of muscle progenitor cells.
48
What are some supportive treatments for Myotonic Dystrophy?
* Modification of food consistency * Physiotherapy * Respiratory support as needed
49
What is Asphyxiating Thoracic Dystrophy also known as?
Jeune syndrome or thoracic-pelvic-phalangeal dystrophy ## Footnote It is the most common hypoplastic thorax syndrome.
50
What type of disorder is Asphyxiating Thoracic Dystrophy?
Autosomal recessive disorder ## Footnote It is part of the ciliary chondrodysplasias.
51
What are the main characteristics of Asphyxiating Thoracic Dystrophy?
* Narrow hypoplastic rib cage * Generalized chondrodystrophy with short limb dwarfism * Pelvic and phalangeal abnormalities * Polydactyly * Renal and hepatic disorders * Thrombocytopenia * Shwachman syndrome ## Footnote Renal disease emerging later in life is a serious concern.
52
What is Jarcho-Levin Syndrome?
An alternative type of chest wall hypoplasia ## Footnote It includes spondylocostal dysostosis and spondylothoracic dysplasia.
53
What genetic mutation is associated with Spondylocostal dysostosis?
Mutations in the DLL3 gene ## Footnote This gene is involved in the Notch pathway during early vertebral development.
54
What is the clinical presentation of Pectus Excavatum?
* Limited ability to exercise * Haller score > 5 indicates mild restrictive defect on lung function * Severe PEx causes compression and displacement of the heart ## Footnote Most do not present symptoms at rest.
55
What is the natural history of scoliosis?
* Curve progression * Cardiopulmonary impairment * Back pain * Cosmetic deformity * Poor balance * Neurological compromise ## Footnote Adults diagnosed with scoliosis before age 8 have a higher mortality rate after age 40.
56
What does the Cobb angle measure?
The angle of displacement on an anterior–posterior spine radiograph ## Footnote It does not correlate well with respiratory functional measurements.
57
What is the mortality rate of untreated Adolescent Idiopathic Scoliosis?
2.2 times greater than controls ## Footnote Deaths generally occur in the 4th or 5th decade of life due to cardiopulmonary insufficiency.
58
What defines Congenital Scoliosis?
Usually progressive with unilateral unsegmented bar and convex hemi-vertebrae ## Footnote Cobb angle > 50–60 degrees is indicative.
59
How is respiratory function tested in Kyphoscoliosis?
* Reduction in chest wall compliance * Impaired lung growth * Decreased inspiratory muscle strength * PFT shows reductions in vital capacity ## Footnote FVC is most sensitive to reductions in thoracic cage size.
60
What is the treatment for Pectus Excavatum?
* Ravitch procedure * Nuss procedure ## Footnote Nuss procedure is minimally invasive and involves retrosternal placement of a curved metal bar.
61
What are the management strategies for Adolescent Idiopathic Scoliosis?
* Early detection before the growth spurt * Bracing for curves > 25 degrees * Surgical fusion for uncontrolled progression ## Footnote Surgical treatment may involve staples to promote growth on the concave side.
62
What are the key features of Jarcho-Levin Syndrome?
* Shortened thoracic height * Congenital scoliosis in spondylocostal dysostosis * Respiratory distress in spondylothoracic dysplasia ## Footnote Lung function in adults reflects severe restrictive changes.
63
What is the significance of the Apnea-Hypopnea Index in children with Early Onset Scoliosis?
Usually increased irrespective of CO2 elevation ## Footnote Due to recurrent oxyhemoglobin desaturation associated with hypopneas.
64
What is the role of titanium connectors in the management of Asphyxiating Thoracic Dystrophy?
To make existing ribs longer ## Footnote Customized devices aim to enlarge the thoracic cavity.
65
What is a common respiratory function test in neuromuscular diseases?
Spirometry ## Footnote Other tests include static lung volumes and peak cough flow.
66
What does a peak cough flow < 160–200 L/min indicate?
Cough is ineffective and risks respiratory infections ## Footnote This is significant in adolescents and adults.
67
What is the general management for children with chest wall dysfunction?
* Adequate nutrition * Correction of electrolytic imbalance * Prevention of respiratory infections ## Footnote Vaccination against pneumococcal and annual flu is recommended.
68
What are the postoperative pulmonary complications following scoliosis surgery?
* Atelectasis * Pneumonia * Pulmonary edema * Respiratory failure ## Footnote These are principal causes of morbidity and mortality in the immediate period after surgery.

Kendigs Respiratory Diseases (38 decks)

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