Developmental Aspects of Lung Disease Flashcards
In what stage of morphogenesis does surfactant being to develop
Saccular stage
Common upper congenital abnormalities
Tracheal anagenesis and stenosis
Tracheomalacia
Tracheo-oesphageal fistula
Common lower congenital abnormalities
Lung anagenesis/pulmonary hypoplasia
Bronchogenic cyst
Congenital pulmonary airway malformation (CPAM)
Congenital diaphragmatic hernia
Presenting features in antenatal scanning
Ultrasound - show up more at 20 weeks scan
Presenting features in newborns
Tachypnoea
Respiratory distress
Feeding issues
Tachypnoea
Abnormally rapid breathing
Presenting features in childhood
Stridor/wheeze
Recurrent pneumonia
Cough
Feeding issues
Asymptomatic presenting features
Incidental finding
Tracheal agenesis
Trachea is absent or underdeveloped
Presentation of tracheal agenesis
Acute respiratory distress at birth
Tracheal stenosis
Tracheal cartilage rings are completed too early, may be generalised or segmental
Time of detection of tracheal stenosis
At birth or within first year
Tracheomalacia
Dynamic abnormal collapse of tracheal walls
Presentation of tracheomalacia
Braking cough
Recurrent croup
Breathless on exertion
Stridor/wheeze
Management of tracheomalacia
Physiotherapy and antibiotics when unwell, natural resolution with time
Causes of tracheomalacia
Genetic conditions
External compression
Tracheo-oesphageal fistula
Abnormal connection between trachea and oesophagus
Presentation of tracheo-oesphageal fistula
Choking
Colour change
Cough with feeding
Unable to pass nasal-gastric tube
Treatment of trachea-oesphageal fistula
Surgical repair (rejoin or elongation)
Complications of trachea-oestophageal fistula
Tracheomalacia
Strictures
Leak
Reflux
Time of detection of trachea-oesphageal fistula
Antenatal or postnatal
Congenital pulmonary airway malformation (CPAM)
Abnormal non-functioning lung tissue, occurs sporadically
Management of CPAM
May resolve spontaneously in utero
Conservative treatment id asymptomatic
Surgical intervention
Time of detection of CPAM
80% detected antenatally
Diaphragm development
Develops from multiple tissue at 7 weeks and closes by 18 weeks
Cause of congenital diaphragmatic hernia
Failure of closure of diaphragm (usually left side)
Time of diagnosis of congenital diaphragmatic hernia
Most antenatally, some cases late
Management of congenital diaphragmatic hernia
Surgical repair - primary closure
Eventration of diaphragm
Abnormal elevation of dome of diaphragm in which all or part of it is largely composed of fibrous tissue
Change in lungs after birth
Lungs inflate and fluid in lungs is absorbed
Transient tachypnoea of newborn
Accumulation of fluid in lungs due to no squeezing of lungs after birth
At which weeks do type II pneumocytes differentiate
Weeks 24-34
What is surfactant made up of
Phospholipids and lipophilic proteins
Other name of RDS
Hyaline membrane disease
What causes RDS
Surfactant deficiency
Treatment of RDS
Antenatal steroids (matures lungs)
Surfactant replacement
Appropriate ventilation and nutrition
Bronchopulmonary dysplasia
Chronic lung disease that results from damage to lungs caused by mechanical ventilation and long term use of oxygen
Remodelling
Alteration of airway structure following external influence
Result of remodelling
Interference of inter-cellular signalling
Causes of remodelling
Environmental influences Chronic diseases in childhood Infection Gene interactions Antenatal programming Early allergy
Remodelling in asthma
Chronic inflammation Increased bronchial responsiveness Increase mucus secretion Airway oedema Airway narrowing
Remodelling in chronic lung disease
Chronic inflammation
Interference of inter-cellular signalling
Treatment toxicity - can cause structural change in airways
Pros of prediction of future lung development
Allows identification of illnesses that will appear later in life and so allows for earlier treatment
