Developmental aspects of Lung Disease Flashcards

1
Q

what forms during the embryology of the respiratory tract

A
  • The respiratory tract, diaphragm and lungs do form early in embryonic development.
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2
Q

describe the stages of embryonic development

A
  • Embryonic 3-8 weeks
  • Pseudo-glandular 5-17 weeks
  • Canalicular 16-26 weeks
  • Saccular 24-38 weeks
  • Alveolar 36 weeks – 2/3 years
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3
Q

what happens during the embryonic stage?

A
  • 3-8 weeks

- formation of trachea and main bronchi and lung buds.

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4
Q

what happens during the pseudo glandular stage?

A
  • 5-17 weeks
  • the spreading of the bronchi where all conducting airways form. The accompanying blood vessels also form, the wall structure and epithelial cells differentiate.
  • Cilia develops at 13 weeks.
  • Bronchi forms at around 8-13 weeks
  • Bronchioli forms at 3-10 weeks
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5
Q

what happens during the canalicular stage?

A
  • 16-26 weeks
  • is where the early gas exchange structures form.
  • The Lungs becomes extremely vascularised.
  • Surfactant production appears. This is the age at which babies can be born.
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6
Q

what happens during the saccular stage?

A
  • 24-38 weeks
  • further evolution of gas exchange structures where saccules and then alveoli appear. Type 1 and Type 2 cells. Pre-cursors to alveoli.
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7
Q

what happens during the alveolar stage?

A
  • 36 weeks- 2/3 years

- usually happens after the child is born and continues to grow over the next 3-12 years.

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8
Q

describe the development of the diaphragm

A
  • which is essential for respiration
  • develops from multiple tissues at around 7 weeks.
  • Innervated by the phrenic nerve.
  • Closure by around 18 weeks.
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9
Q

what are the common congenital anomalies affecting the respiratory tract?

A

Upper (Tracheo-Bronchial):

  • Tracheal agenesis and stenosis
  • Tracheomalacia
  • Tracheo-oesophageal fistula

Lower (broncho-pulmonary):

  • Lung agensis/pulmonary hypoplasia
  • Bronchogenic cyst
  • CPAM
  • Congenital Diaphragmatic Hernia
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10
Q

tracheal Agenesis

A
  • Presents at birth, the trachea is completely Absent

- acute respiratory distress and inability to intubate

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11
Q

tracheal stenosis

A
  • presents at birth or within the first year
  • tracheal cartilage rings are complete too early, and are fixed.
  • NARROWING of the trachea
  • may be generalised or segmental
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12
Q

Tracheomalacia

A
  • “Softening”. Floppiness of the airways
  • flaccidity of tracheal cartilage
  • Dynamic abnormal collapse of tracheal walls.
  • May be caused by external compression
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13
Q

what is the presentation and management of tracheomalcia?

A

Presentation:

  • Barking cough
  • Stridor/wheeze

Management:
- physio and antibiotics.

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14
Q

Tracheo-oesophageal fistula

A
  • ABNORMAL connection between trachea and oesophagus
  • Associated with genetic conditions
  • Can be diagnosed antenatally or postnatally
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15
Q

presentation, treatment and complications of trachea-oesophageal fistula?

A

Presentation:

  • Choking
  • Colour change
  • Cough with feeding
  • Unable to pass a nasogastric tube

Treatment:
- Surgical repair

Complications:
- Prone to leaks around the site, reflux, tracheomalacia

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16
Q

LOWER (broncho pulmonary conditions)

Congenital pulmonary airway malformation (CPAM)

A
  • Cystic mass of abnormal lung tissue.
  • Abnormal non-functioning lung tissue
  • 80% is detected antenatally
  • Occurs sporadically
  • May resolve spontaneously in utero
  • Conservative management if asymptomatic
  • Possible risk of malignant change
17
Q

Congenital Diaphragmatic hernia

A
  • A defect in the diaphragm.
  • The diaphragm separates the organs in the abdomen from those in the chest
  • There are different types, most common one is Bochdalek (90%)
  • Usually the left side rather than the right side
  • Most are diagnosed antenatall
18
Q

management and prognosis of congenital diaphragmatic hernia

A

Management: surgical repair

Prognosis depends on the degree of lung hypoplasia

19
Q

Presenting features of anomalies affecting the respiratory tract

A
  • Antenatal screening (ultrasound)
  • Feotal abnormality scan at around 20 weeks
  • New born:
    o Tachypnoea (suggest resp system is impaired)
    o Respiratory distress
    o Feeding issues
-	Childhood:
o	Recurrent pneumonia (croup the infection of upper airways which suggests a structural abnormality)
o	Stridor (inspiratory sucking in sound)
o	Wheeze
o	Feeding issues
  • Asymptomatic:
    o Incidental finding
20
Q

describe the changes in the lung after birth

A
  • Significant changes occur at birth after the babies first breath
  • The lungs inflate and fluid in the lungs is ABSORBED
  • Transient tachyponoea of newborn:
    o Associated with C SECTION
    o but it Improves within 1-2 days
21
Q

when does surfactant production begin?

A
  • during the canicular stage (16-26)
  • type 2 pneumocytes differentiate at 24 -34 weeks

(reminder than surface cells reduce surface tension, promoting gas exchange)

22
Q

what can occur in preterm infants with surfactant deficiency (hyaline membrane disease)?

describe the treatment

A
  • Respiratroy distress syndrome
  • Antenatal steroids
  • Surfactant replacement
  • Appropriate ventilation and nutrition
23
Q

Understand the relationship between respiratory illness in childhood

A
  • Antenatal:
  • Nicotine exposure from mother
  • Mothers nutrition
  • Mothers micronutrients/ vitamins
  • Infection
  • Low weight at birth/premature
  • Antenatal steroids
24
Q

Understand the relationship between respiratory illness in adulthood

A
  • Post natal
  • Early life events reduce normal growth and lung function
  • Barker hypothesis
  • Infection
  • Growth
  • Tobacco exposure
  • Environmental pollution
  • Micronutrients/vitamins

-May lead to the risk of future COPD

25
Q

what is remodelling

A
  • Aspects of the airways and lungs change because of external influences:
  • Environmental exposure
  • Early allergy and gene interactions
  • Chronic diseases of childhood
  • Infection
26
Q

causes of remodelling

A

• Interference of inter-cellular signalling

27
Q

where is remodelling seen?

A

-Asthma

  • wider lumen, significant changes in surface and interstially
  • Chronic inflammation
  • Increased bronchial responsiveness
  • Increase mucus secretion
  • Airway oedema
  • Airway narrowing

-Chronic lung disease:

  • Chronic inflammation
  • Interference inter-cellular signalling
  • Treatment toxicity
28
Q

U_n_d_e_r_s_t_a_n_d_ t_h_e “t_r_a_c_k_i_n_go_f p_u_l_m_o_n_a_r_y f_u_n_c_t_i_o_n f_r_o_m e_a_r_l_y l_i_f_e i_n_t_o a_d_u_l_t_h_o_o_d _and factors which influence tracking

A
  • Early lung function tracks in adulthood
  • Stopping smoking decreases pulmonary lung function
  • Premature births peak to a lower baseline of FEV and fall earlier on