Developmental Aspects of Lung Disease Flashcards

1
Q

What are the different stages of lung development?

A

Embryonic (3-8wks) - bronchi form.
Pseudoglandular (5-17wks) - bronchioles form.
Canalicular (16-26wks) - respiratory portion forms.
Saccular (36wks-3yrs) - alveolar ducts form, surfactant production occurs.
Alveolar (3-8yrs) - alveolar sacs form.

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2
Q

What are the investigations for antenatal screening?

A

US.
MRI.

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3
Q

What symptoms suggest developmental lung disease?

A

Newborn - tachypnoea, respiratory distress, feeding issues.
Childhood - stridor, wheeze, recurrent pneumonia, cough, feeding issues.
Asymptomatic - incidental finding.

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4
Q

What is laryngomalacia?

A

A common upper congenital abnormality in infants; improves within the first year.
Presentation - stridor, worse with feeding / upset / excited.
Concern if it affects feeding, growth, or causes apnoeas.

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5
Q

What is tracheomalacia?

A

An upper congenital abnormality that can be isolated in healthy infants.
Associated with genetic conditions.
May be caused by external compression from vessels or tumours.

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6
Q

What is the presentation, management and progression of tracheomalacia?

A

Presentation - barking cough, recurrent ‘croup’, breathless on exertion, stridor, wheeze.
Management - physio, abx when unwell.
Progression - resolves with time.

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7
Q

What is a TOF?

A

An upper congenital abnormal connection between the trachea and oesophagus; is associated with genetic conditions.

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8
Q

What are the diagnosis, presentation, treatment and complications of TOF?

A

Diagnosis - antenatally or postnatally.
Presentation - choking, colour change, cough with feeding, unable to pass NG.
Treatment - surgical repair.
Complications - tracheomalacia, strictures, leak and reflux.

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9
Q

What is CPAM?

A

Congenital Pulmonary Airway Malformation.
A lower congenital abnormality - non-functioning lung tissue that occurs sporadically.
80% of cases are detected antenatally.

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10
Q

What is the progression, management and treatment of CPAM?

A

Progression - may resolve spontaneously in utero (possible risk of malignant change).
Management - conservative, if asymptomatic.
Treatment - surgical intervention (if required).

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11
Q

What is a congenital diaphragmatic hernia?

A

A lower congenital abnormality - the tissues around the diaphragm do not close properly.
Different types (Bochdalek = 90% of cases).
Usually the left side is affected.

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12
Q

What are the diagnosis, management and prognosis of a congenital diaphragmatic hernia?

A

Diagnosis - mostly antenatal.
Management - surgical repair.
Prognosis - depends on the degree of lung hypoplasia.

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13
Q

What is transient tachypnoea of newborns?

A

Absorption of fluids into the lungs on inflation on the first breath.
Associated with C-sections.
Improves within 1-2 days.

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14
Q

What is Respiratory Distress Syndrome?

A

In preterm infants and some term infants due to surfactant deficiency.
Also called hyaline membrane disease.

Treatment - antenatal steroids, surfactant replacement, ventilation and nutrition.

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15
Q

What is chronic lung disease?

A

Associated with prematurity where ongoing oxygen requirement is at term, and increased childhood respiratory morbidity.
Also called bronchopulmonary dysplasia.
Multifactorial causes.
Leads to chronic obstructions in later life.

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16
Q

What relationships between paediatric and adult lung disease exist?

A

Individual - sex, age, height.
Early life events - parents, smoking, birth weight, breastfeeding, lung infections.
Environment - pollution, BMI.
Allergies - asthma, rhinitis, sensitivities.

17
Q

What is remodelling?

A

The alteration of airway structure following external influence.
Caused by environmental exposures, chronic diseases of childhood, or infection.

18
Q

What physiological changes occur in remodelling?

A

Abnormalities due to the interference of inter-cellular signalling.
Eosinophilia, vasodilation, collagen deposition.
Oedema, hypertrophy, desquamation.

19
Q

What causes remodelling?

A

Asthma.
Chronic lung disease.
Chronic inflammation - increased bronchial responsiveness and mucus secretion.

20
Q

What factors affect lung function growth?

A

Low - genetics, preterm birth, early exposures, LRTIs, childhood asthma.
High - genetics, exposure avoidance, diet, physical activity, medicine.