Podcast
Questions and Answers
What is the primary characteristic of congenital arrhinia?
What is the primary characteristic of congenital arrhinia?
- Presence of two fully developed noses
- Presence of a rudimentary nasal structure
- Underdevelopment of the nasal cavity structures (correct)
- Incomplete formation of the mouth
Which condition involves complete duplication of the nose?
Which condition involves complete duplication of the nose?
- Proboscis lateralis
- Nasal hypoplasia
- Polyrhinia (correct)
- Congenital arrhinia
What is a potential cause of congenital arrhinia?
What is a potential cause of congenital arrhinia?
- Excessive development of nasal placodes
- Overgrowth of neural crest cells
- Abnormal fusion of the medial nasal processes (correct)
- Improper development of the pharyngeal arches
At what age is reconstructive surgery typically performed for congenital arrhinia?
At what age is reconstructive surgery typically performed for congenital arrhinia?
What is the primary treatment for a condition characterized by a rudimentary nasal structure off-center from the midline?
What is the primary treatment for a condition characterized by a rudimentary nasal structure off-center from the midline?
What is NOT involved in the components of the pharyngeal arches?
What is NOT involved in the components of the pharyngeal arches?
What does the first pharyngeal cleft become in development?
What does the first pharyngeal cleft become in development?
What is the primary function of pharyngeal arch 2?
What is the primary function of pharyngeal arch 2?
Which membrane separates the stomodeum from the primordial pharynx?
Which membrane separates the stomodeum from the primordial pharynx?
Which structure is formed from the first pharyngeal pouch?
Which structure is formed from the first pharyngeal pouch?
What is pulmonary agenesis characterized by?
What is pulmonary agenesis characterized by?
What is the primary etiology associated with pulmonary aplasia?
What is the primary etiology associated with pulmonary aplasia?
What is a common symptom of pulmonary hypoplasia?
What is a common symptom of pulmonary hypoplasia?
Which factor is NOT associated with neonatal respiratory distress syndrome?
Which factor is NOT associated with neonatal respiratory distress syndrome?
What treatment can enhance fetal lung maturation in cases of pulmonary hypoplasia?
What treatment can enhance fetal lung maturation in cases of pulmonary hypoplasia?
Which symptom is common in neonates suffering from respiratory distress syndrome?
Which symptom is common in neonates suffering from respiratory distress syndrome?
Which of the following statements about surfactant is correct?
Which of the following statements about surfactant is correct?
What is the expected outcome for most infants with mild symptoms of neonatal respiratory distress syndrome?
What is the expected outcome for most infants with mild symptoms of neonatal respiratory distress syndrome?
What condition is characterized by an undersized lower jaw due to abnormal development of the first pharyngeal pouch?
What condition is characterized by an undersized lower jaw due to abnormal development of the first pharyngeal pouch?
Which genetic abnormality is associated with DiGeorge syndrome?
Which genetic abnormality is associated with DiGeorge syndrome?
What treatment is NOT typically recommended for DiGeorge syndrome?
What treatment is NOT typically recommended for DiGeorge syndrome?
What symptoms are included in the CATCH acronym for DiGeorge syndrome?
What symptoms are included in the CATCH acronym for DiGeorge syndrome?
Which treatment is most crucial for resolving feeding problems associated with cleft palate in DiGeorge syndrome?
Which treatment is most crucial for resolving feeding problems associated with cleft palate in DiGeorge syndrome?
What is a primary function of the larynx?
What is a primary function of the larynx?
How does the structure of the laryngeal orifice change during development?
How does the structure of the laryngeal orifice change during development?
Which cartilage encircles the trachea completely?
Which cartilage encircles the trachea completely?
What primarily induces the formation of the laryngeal ventricles?
What primarily induces the formation of the laryngeal ventricles?
What is the major developmental origin of the laryngeal epithelium?
What is the major developmental origin of the laryngeal epithelium?
What is the most common anomaly of the lower respiratory tract?
What is the most common anomaly of the lower respiratory tract?
Which of the following is a potential complication of tracheoesophageal fistula?
Which of the following is a potential complication of tracheoesophageal fistula?
What is a common symptom of tracheoesophageal fistula?
What is a common symptom of tracheoesophageal fistula?
What is the primary method of treatment for tracheoesophageal fistula?
What is the primary method of treatment for tracheoesophageal fistula?
What is a cause of tracheoesophageal fistula?
What is a cause of tracheoesophageal fistula?
What defines tracheal stenosis?
What defines tracheal stenosis?
How frequently does tracheoesophageal fistula occur in live births?
How frequently does tracheoesophageal fistula occur in live births?
What can result from the reflux of gastric contents through the fistula?
What can result from the reflux of gastric contents through the fistula?
What is the relationship between tracheoesophageal fistula and esophageal atresia?
What is the relationship between tracheoesophageal fistula and esophageal atresia?
What results from unequal partitioning of the foregut?
What results from unequal partitioning of the foregut?
What primary function does the respiratory system NOT perform?
What primary function does the respiratory system NOT perform?
During which week of embryonic development does the respiratory system begin to form?
During which week of embryonic development does the respiratory system begin to form?
The laryngotracheal groove is primarily a precursor to which of the following structures?
The laryngotracheal groove is primarily a precursor to which of the following structures?
What induces the bud formation and lung growth within the respiratory diverticulum?
What induces the bud formation and lung growth within the respiratory diverticulum?
What type of tissue primarily gives rise to the connective tissue and cartilage of the lungs?
What type of tissue primarily gives rise to the connective tissue and cartilage of the lungs?
Which cavity forms as the nasal pits deepen during development?
Which cavity forms as the nasal pits deepen during development?
What structure separates the nasal sacs from the oral cavity during development?
What structure separates the nasal sacs from the oral cavity during development?
What results from the apoptosis of an epithelial plug during nasal development?
What results from the apoptosis of an epithelial plug during nasal development?
The primordial choanae are located at the junction between which two structures?
The primordial choanae are located at the junction between which two structures?
The nasal placodes are considered to be which type of embryonic structure?
The nasal placodes are considered to be which type of embryonic structure?
Which embryonic layer gives rise to the pulmonary epithelium and glands of the respiratory system?
Which embryonic layer gives rise to the pulmonary epithelium and glands of the respiratory system?
What embryonic structure is described as an outpouching of the foregut?
What embryonic structure is described as an outpouching of the foregut?
Which embryonic structure develops from the maxillary prominences?
Which embryonic structure develops from the maxillary prominences?
What is the incorrect association regarding the origins of various respiratory organs?
What is the incorrect association regarding the origins of various respiratory organs?
What is bronchial atresia characterized by?
What is bronchial atresia characterized by?
What is the primary cause of bronchial atresia?
What is the primary cause of bronchial atresia?
Which diagnostic tools are used to identify bronchial atresia?
Which diagnostic tools are used to identify bronchial atresia?
What are possible symptoms of bronchial atresia?
What are possible symptoms of bronchial atresia?
What complication can arise due to bronchial atresia?
What complication can arise due to bronchial atresia?
Which stage of lung development involves the formation of terminal sacs?
Which stage of lung development involves the formation of terminal sacs?
At what stage does respiration become possible for fetuses?
At what stage does respiration become possible for fetuses?
What type of bronchi is referred to as tertiary bronchi?
What type of bronchi is referred to as tertiary bronchi?
What is a primary function of type II pneumocytes?
What is a primary function of type II pneumocytes?
Which developmental stage has no respiratory bronchioles or alveoli present?
Which developmental stage has no respiratory bronchioles or alveoli present?
When does the production of surfactant begin to occur?
When does the production of surfactant begin to occur?
What are the factors essential for normal lung development?
What are the factors essential for normal lung development?
How is fluid cleared from the lungs at birth?
How is fluid cleared from the lungs at birth?
During which stage do the terminal sacs form and establish gas-exchanging capabilities?
During which stage do the terminal sacs form and establish gas-exchanging capabilities?
What is a common treatment for polyrhinia?
What is a common treatment for polyrhinia?
Which abnormality is characterized by a rudimentary nasal structure positioned off-center?
Which abnormality is characterized by a rudimentary nasal structure positioned off-center?
Which of the following is a proposed etiology for congenital arrhinia?
Which of the following is a proposed etiology for congenital arrhinia?
At what age is reconstructive surgery typically recommended for congenital arrhinia?
At what age is reconstructive surgery typically recommended for congenital arrhinia?
What primary structure does the first pharyngeal pouch develop into?
What primary structure does the first pharyngeal pouch develop into?
What is the role of the pharyngeal arches during embryonic development?
What is the role of the pharyngeal arches during embryonic development?
How many pharyngeal arches are involved in the development of the human face and neck?
How many pharyngeal arches are involved in the development of the human face and neck?
What is one of the primary functions of the second pharyngeal arch?
What is one of the primary functions of the second pharyngeal arch?
What distinguishes congenital arrhinia from other nasal congenital abnormalities?
What distinguishes congenital arrhinia from other nasal congenital abnormalities?
What characterizes pulmonary agenesis?
What characterizes pulmonary agenesis?
Which of the following complications is associated with pulmonary hypoplasia?
Which of the following complications is associated with pulmonary hypoplasia?
What is a common etiology associated with pulmonary aplasia?
What is a common etiology associated with pulmonary aplasia?
Which symptom is associated with neonatal respiratory distress syndrome?
Which symptom is associated with neonatal respiratory distress syndrome?
What treatment is typically used for improving fetal lung maturity?
What treatment is typically used for improving fetal lung maturity?
What is a major symptom of impaired surfactant production in newborns?
What is a major symptom of impaired surfactant production in newborns?
What is the expected outcome for most infants with neonatal respiratory distress syndrome?
What is the expected outcome for most infants with neonatal respiratory distress syndrome?
What changes occur in the lungs due to prolonged intrauterine asphyxia in newborns?
What changes occur in the lungs due to prolonged intrauterine asphyxia in newborns?
What is micrognathia primarily characterized by?
What is micrognathia primarily characterized by?
Which syndrome is associated with a microdeletion at chromosome 22q11.2?
Which syndrome is associated with a microdeletion at chromosome 22q11.2?
Which treatment is NOT commonly used for managing DiGeorge syndrome?
Which treatment is NOT commonly used for managing DiGeorge syndrome?
What does the CATCH acronym stand for in relation to DiGeorge syndrome symptoms?
What does the CATCH acronym stand for in relation to DiGeorge syndrome symptoms?
What is the primary role of the larynx in the respiratory system?
What is the primary role of the larynx in the respiratory system?
What developmental event changes the appearance of the laryngeal orifice from a sagittal slit to a T-shaped opening?
What developmental event changes the appearance of the laryngeal orifice from a sagittal slit to a T-shaped opening?
Which cartilage structure is responsible for fully encircling the trachea?
Which cartilage structure is responsible for fully encircling the trachea?
Which complication is NOT typically associated with DiGeorge syndrome?
Which complication is NOT typically associated with DiGeorge syndrome?
What initiates the laryngeal ventricles' formation during development?
What initiates the laryngeal ventricles' formation during development?
What is the most common variety of tracheoesophageal fistula?
What is the most common variety of tracheoesophageal fistula?
What is an essential outcome of the rapid proliferation of epithelial tissue in the larynx development?
What is an essential outcome of the rapid proliferation of epithelial tissue in the larynx development?
What is a primary symptom of tracheoesophageal fistula in infants?
What is a primary symptom of tracheoesophageal fistula in infants?
Which condition is associated with tracheoesophageal fistula?
Which condition is associated with tracheoesophageal fistula?
What is the recommended treatment for tracheoesophageal fistula?
What is the recommended treatment for tracheoesophageal fistula?
Which of the following best describes a tracheoesophageal fistula?
Which of the following best describes a tracheoesophageal fistula?
What causes the development of tracheoesophageal fistula?
What causes the development of tracheoesophageal fistula?
What can excessive liquid accumulation in the mouth and upper respiratory tract indicate?
What can excessive liquid accumulation in the mouth and upper respiratory tract indicate?
What type of condition does tracheal stenosis represent?
What type of condition does tracheal stenosis represent?
How often does tracheoesophageal fistula occur in live births?
How often does tracheoesophageal fistula occur in live births?
What is a potential consequence of gastric contents refluxing into the trachea through a fistula?
What is a potential consequence of gastric contents refluxing into the trachea through a fistula?
What is the primary role of the respiratory diverticulum during embryonic development?
What is the primary role of the respiratory diverticulum during embryonic development?
Which embryonic layer contributes to the pulmonary epithelium and glands of the respiratory system?
Which embryonic layer contributes to the pulmonary epithelium and glands of the respiratory system?
What occurs during the 6th week of nasal cavity development?
What occurs during the 6th week of nasal cavity development?
Which structure forms as an outpouching of the hollow foregut?
Which structure forms as an outpouching of the hollow foregut?
What anatomical structures are primarily formed from the splanchnic mesoderm?
What anatomical structures are primarily formed from the splanchnic mesoderm?
Which transcription factor is upregulated by retinoic acid during lung development?
Which transcription factor is upregulated by retinoic acid during lung development?
What occurs at the end of week 4 in the development of the respiratory system?
What occurs at the end of week 4 in the development of the respiratory system?
Which structure develops from the nasal placodes during embryogenesis?
Which structure develops from the nasal placodes during embryogenesis?
What predominantly influences bud formation and lung growth in the respiratory diverticulum?
What predominantly influences bud formation and lung growth in the respiratory diverticulum?
Which anatomical feature is formed from the apoptosis of an epithelial plug during nasal development?
Which anatomical feature is formed from the apoptosis of an epithelial plug during nasal development?
Which is a function of the respiratory system?
Which is a function of the respiratory system?
What does the laryngotracheal groove give rise to in embryonic development?
What does the laryngotracheal groove give rise to in embryonic development?
What defines the nasal vestibule's formation during development?
What defines the nasal vestibule's formation during development?
What is the expected outcome of the primordial nasal sacs growing into the space underneath the forebrain?
What is the expected outcome of the primordial nasal sacs growing into the space underneath the forebrain?
What is the primary symptom associated with bronchial atresia?
What is the primary symptom associated with bronchial atresia?
Which type of bronchial atresia involves a focal interruption of a segmental or subsegmental bronchus?
Which type of bronchial atresia involves a focal interruption of a segmental or subsegmental bronchus?
What can follow bronchial atresia due to obstruction in a lung segment?
What can follow bronchial atresia due to obstruction in a lung segment?
What imaging technique is typically used for diagnosing bronchial atresia?
What imaging technique is typically used for diagnosing bronchial atresia?
What treatment is recommended for bronchial atresia?
What treatment is recommended for bronchial atresia?
What stage of lung development occurs between weeks 16-26 of gestation?
What stage of lung development occurs between weeks 16-26 of gestation?
During which stage of lung development are terminal sacs (primitive alveoli) formed?
During which stage of lung development are terminal sacs (primitive alveoli) formed?
Which type of bronchi is classified as tertiary bronchi?
Which type of bronchi is classified as tertiary bronchi?
What physiological change is necessary for successful respiration after birth?
What physiological change is necessary for successful respiration after birth?
Which type of bronchial niche complicates bronchial atresia due to mucus buildup?
Which type of bronchial niche complicates bronchial atresia due to mucus buildup?
What developmental factor is essential for normal lung growth?
What developmental factor is essential for normal lung growth?
At what gestational weeks does the respiratory diverticulum begin its development?
At what gestational weeks does the respiratory diverticulum begin its development?
When does the production of pulmonary surfactant begin?
When does the production of pulmonary surfactant begin?
Which structure forms from the differentiation of terminal sacs during lung development?
Which structure forms from the differentiation of terminal sacs during lung development?
Flashcards
Respiratory system development
Respiratory system development
The process of forming the parts of the respiratory system, beginning in week 4 of development.
Laryngotracheal groove
Laryngotracheal groove
An indentation in the floor of the pharynx that gives rise to parts of the respiratory system.
Respiratory diverticulum
Respiratory diverticulum
A pouch-like outgrowth from the laryngotracheal groove.
Endoderm origin
Endoderm origin
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Mesoderm origin
Mesoderm origin
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Nasal placodes
Nasal placodes
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Nasal Pits
Nasal Pits
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Oronasal membrane
Oronasal membrane
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Nasal vestibule
Nasal vestibule
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Primordial choanae
Primordial choanae
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Olfactory epithelium
Olfactory epithelium
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Olfactory receptor cells
Olfactory receptor cells
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Facial prominences
Facial prominences
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Frontonasal prominence
Frontonasal prominence
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Tbx4
Tbx4
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Congenital arrhinia
Congenital arrhinia
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Polyrhinia
Polyrhinia
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Proboscis lateralis
Proboscis lateralis
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Pharyngeal arches
Pharyngeal arches
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Pharyngeal pouches
Pharyngeal pouches
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Pharyngeal clefts
Pharyngeal clefts
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Pharyngeal membranes
Pharyngeal membranes
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Pharyngeal arch artery
Pharyngeal arch artery
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Pharyngeal arch cartilage
Pharyngeal arch cartilage
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Pharyngeal arch muscles
Pharyngeal arch muscles
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Micrognathia
Micrognathia
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Pierre Robin syndrome
Pierre Robin syndrome
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DiGeorge syndrome
DiGeorge syndrome
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22q11.2 deletion
22q11.2 deletion
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Laryngeal orifice
Laryngeal orifice
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Laryngeal lumen
Laryngeal lumen
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Vocal folds
Vocal folds
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Thyroid cartilage
Thyroid cartilage
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Tracheoesophageal fistula
Tracheoesophageal fistula
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Esophageal atresia
Esophageal atresia
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What causes tracheoesophageal fistula?
What causes tracheoesophageal fistula?
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How does tracheoesophageal fistula affect swallowing?
How does tracheoesophageal fistula affect swallowing?
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Bifurcation
Bifurcation
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Tracheal stenosis
Tracheal stenosis
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Tracheal atresia
Tracheal atresia
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How are tracheal stenosis and atresia treated?
How are tracheal stenosis and atresia treated?
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Primary bronchial buds
Primary bronchial buds
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Secondary and tertiary bronchial buds
Secondary and tertiary bronchial buds
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Pulmonary agenesis
Pulmonary agenesis
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Pulmonary aplasia
Pulmonary aplasia
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Pulmonary hypoplasia
Pulmonary hypoplasia
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Neonatal respiratory distress syndrome (NRDS)
Neonatal respiratory distress syndrome (NRDS)
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Surfactant
Surfactant
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What causes NRDS?
What causes NRDS?
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NRDS Symptoms
NRDS Symptoms
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NRDS Treatment
NRDS Treatment
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Bronchial Atresia
Bronchial Atresia
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Proximal Bronchial Atresia
Proximal Bronchial Atresia
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Peripheral Bronchial Atresia
Peripheral Bronchial Atresia
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Etiology of Bronchial Atresia
Etiology of Bronchial Atresia
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Symptoms of Bronchial Atresia
Symptoms of Bronchial Atresia
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Complications of Bronchial Atresia
Complications of Bronchial Atresia
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Diagnosis of Bronchial Atresia
Diagnosis of Bronchial Atresia
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Treatment for Bronchial Atresia
Treatment for Bronchial Atresia
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Embryonic Stage of Lung Development
Embryonic Stage of Lung Development
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Pseudoglandular Stage of Lung Development
Pseudoglandular Stage of Lung Development
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Canalicular Stage of Lung Development
Canalicular Stage of Lung Development
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Saccular Stage of Lung Development
Saccular Stage of Lung Development
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Alveolar Stage of Lung Development
Alveolar Stage of Lung Development
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Type I Pneumocytes
Type I Pneumocytes
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Type II Pneumocytes
Type II Pneumocytes
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What is the main function of the respiratory system?
What is the main function of the respiratory system?
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What germ layer forms the pulmonary epithelium and glands?
What germ layer forms the pulmonary epithelium and glands?
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What germ layer forms the cartilage and smooth muscle of the respiratory system?
What germ layer forms the cartilage and smooth muscle of the respiratory system?
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What is the respiratory diverticulum?
What is the respiratory diverticulum?
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What is the role of retinoic acid in lung development?
What is the role of retinoic acid in lung development?
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What are the five facial primordia?
What are the five facial primordia?
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What are nasal placodes?
What are nasal placodes?
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How does the nasal cavity develop?
How does the nasal cavity develop?
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What happens when the oronasal membrane ruptures?
What happens when the oronasal membrane ruptures?
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What is the role of the olfactory epithelium?
What is the role of the olfactory epithelium?
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What is the effect of the development and apoptosis of an epithelial plug?
What is the effect of the development and apoptosis of an epithelial plug?
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What are the potential developmental issues with the nasal cavity?
What are the potential developmental issues with the nasal cavity?
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What is the pharynx formed from?
What is the pharynx formed from?
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What are potential defects in the development of the pharynx?
What are potential defects in the development of the pharynx?
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What is the laryngeal orifice?
What is the laryngeal orifice?
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What does each pharyngeal arch contain?
What does each pharyngeal arch contain?
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What are the six pharyngeal arches?
What are the six pharyngeal arches?
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Pharyngeal Derivatives
Pharyngeal Derivatives
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What is micrognathia?
What is micrognathia?
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What causes micrognathia?
What causes micrognathia?
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What happens during a Laryngeal Orifice Transformation?
What happens during a Laryngeal Orifice Transformation?
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What is the role of the thyroid cartilage?
What is the role of the thyroid cartilage?
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What are the functions of the larynx?
What are the functions of the larynx?
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What are the origins of the larynx?
What are the origins of the larynx?
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How are the vocal folds formed?
How are the vocal folds formed?
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What is the purpose of the epiglottis?
What is the purpose of the epiglottis?
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Most common anomaly of the lower respiratory tract
Most common anomaly of the lower respiratory tract
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Symptoms of tracheoesophageal fistula
Symptoms of tracheoesophageal fistula
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Treatment for tracheoesophageal fistula
Treatment for tracheoesophageal fistula
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Treatment for tracheal stenosis
Treatment for tracheal stenosis
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Secondary bronchi
Secondary bronchi
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Tertiary bronchial buds
Tertiary bronchial buds
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Bronchial Atresia Etiology
Bronchial Atresia Etiology
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Bronchial Atresia Symptoms
Bronchial Atresia Symptoms
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Bronchial Atresia Complications
Bronchial Atresia Complications
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Bronchial Atresia Diagnosis
Bronchial Atresia Diagnosis
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Bronchial Atresia Treatment
Bronchial Atresia Treatment
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Study Notes
Embryonic Development of the Respiratory System
- The respiratory system develops from the laryngotracheal groove in week 4 of development.
- The tracheobronchial tree forms caudal to the fourth pair of pharyngeal pouches.
- The respiratory diverticulum is the primitive form of the respiratory system, emerging from the ventral wall of the foregut.
- The respiratory diverticulum grows out from the laryngotracheal groove and forms a pouch-like structure.
Main Functions of the Respiratory System
- Respiration: The primary function of respiration is the exchange of gases.
- Smell: The external nose detects scents.
- Filtration/defense: The respiratory system filters and removes foreign particles.
- Sound & speech production: The larynx aids in producing sounds and speech.
- Elimination of waste: The respiratory system gets rid of waste materials in the form of gases.
Origins of Respiratory System Organs
- Endoderm: Provides the pulmonary epithelium and glands.
- Splanchnic Mesoderm: Provides connective tissue, cartilage, and smooth muscle components.
Development of the Nasal Cavities
- Nasal placodes develop by the end of the 4th week.
- These placodes become depressed, forming the nasal pits.
- Surrounding mesenchyme proliferates into the medial and lateral nasal prominences.
- The pits deepen and form primordial nasal sacs.
- The oronasal membrane separates the nasal sacs from the oral cavity.
- The oronasal membrane ruptures in week 6, allowing the oral and nasal cavities to communicate.
- An epithelial plug develops and later disappears, shaping the nasal vestibule.
- The primordial choanae are the regions of continuity between the nasal and oral cavities.
- The primary palate forms the secondary palate.
- Ectodermal epithelium specializes into olfactory epithelium, producing olfactory receptor cells.
Abnormal Development of the Nasal Cavity
- Congenital arrhinia: Lack of nasal structure formation, affects less than 1 in a million births. Possible causes include lack of invagination of nasal placodes, premature medial nasal process fusion, or failure of nasal epithelial plug resorption, or abnormal migration of neural crest cells. Management involves breathing through the mouth and a tracheostomy tube. Treatment usually includes reconstructive surgery at around 5-6 years old.
- Polyrhinia: Complete nose duplication. Treatment includes surgical correction/excision in the medial part of each nose and union of the lateral halves.
- Proboscis lateralis: Rudimentary nasal structure, off-center from the face midline. Treatment: Heminose reconstruction.
Development of the Pharyngeal Apparatus
- The pharyngeal apparatus comprises arches, pouches, grooves/clefts, and membranes.
- The pharyngeal arches develop neural crest cells into the head and neck regions.
- Each arch contains mesenchymal tissue, covered externally by ectoderm and internally by endoderm.
- The arches are separated by pharyngeal grooves/clefts.
- The pharyngeal membranes form at the points where the arches' epithelia contact.
- The pharyngeal arches contribute significantly to the development of the face, nasal cavities, mouth, larynx, pharynx, and neck.
Abnormal Development of the Pharynx - Micrognathia
- Also known as mandibular hypoplasia or undersized lower jaw.
- Potential etiologies include Pierre Robin syndrome, Trisomy 13 (Patau syndrome), and Fetal alcohol syndrome
- Symptoms: Feeding or breathing problems and teeth malocclusion.
- Treatment: Includes corrective surgery and orthodontic braces.
Abnormal Development of the Pharynx - DiGeorge Syndrome
- Microdeletion of chromosome 22 at 22q11.2.
- Symptoms (CATCH): Cardiac anomalies, Abnormal facies, Thymic hypoplasia or aplasia, Cleft palate, and Hypocalcemia.
- Diagnosis: Genetic testing, X-rays, CT scans (looking for cardiac defects), and physical exam.
- Complications include autoimmune disorders, impaired hearing/vision, learning disabilities, and feeding problems.
- Treatment: Antibiotic medications, calcium supplementation, ear tubes/hearing aids, occupational/physical therapy, and hormone replacement therapy. Surgery can address heart defects and cleft palate.
Development of the Larynx
- The larynx forms from the pharyngeal apparatus.
- Its function includes breathing, sound production, and preventing foreign matter from entering the respiratory system.
- The lining of the larynx originates from endoderm.
- The cartilages and muscles arise from the mesenchyme of the 4th and 6th pharyngeal arches.
- The respiratory primordium connects to the pharynx via the laryngeal orifice.
- The rapid proliferation of mesenchyme forms swellings (e.g., arytenoid swellings); the epithelium (pharyngeal pouch) develops and creates a temporary closure of the laryngeal lumen.
- The laryngeal orifice is crucial for the respiratory primordium and pharynx communication.
Abnormal Development of the Larynx: Laryngeal Atresia
- A rare birth defect.
- The larynx fails to open during intrauterine development.
- No recanalization occurs by week 10 of gestation. The larynx gets obstructed by cartilage.
- Symptoms include congenital high airway obstruction syndrome, dilated airways, hyperplastic lungs, heart/vessel compression, and a flattened diaphragm.
- Diagnosis involves prenatal ultrasonography.
- Treatment involves immediate tracheostomy at birth.
Abnormal Development of the Larynx - Laryngomalacia
- Congenital laryngeal stridor, with an M:F ratio of 2:1.
- Possible causes include congenital malformations in the larynx, abnormal laryngeal cartilage flaccidity, or delayed neuromuscular development with laryngeal hypotonia leading to anatomical abnormality.
- Symptoms include inspiratory stridor (high-pitched wheezing), prolonged feeding time, emesis, choking, coughing, and weight loss.
- Treatment options include acid reflux suppression, feeding modulation, upright posture, supraglottoplasty, and tracheostomy.
Development of the Trachea
- The trachea develops from the respiratory diverticulum.
- The tracheoesophageal ridges separate it from the foregut.
- The ridges fuse to create the tracheoesophageal septum, dividing the foregut into the esophagus and trachea.
- The epithelium of the trachea forms from the endoderm, along with the glands.
- The mesenchyme tissue surrounding the trachea contributes to the connective tissue, cartilage, and muscle.
Abnormal Development of the Trachea - Tracheoesophageal Fistula
- An abnormal opening between the trachea and the esophagus.
- The most common type involves a blind-ending esophagus connected near the trachea's bifurcation.
- Etiology associated with incomplete division of the crania foregut into esophageal and respiratory parts, a failure of the esophageal ridges' fusion, and a defective tracheoesophageal septum.
- Symptoms include accumulation of fluid in the mouth, upper respiratory tract, cough and choking, and regurgitation due to reflux of gastric content into the trachea and lungs.
- Treatment involves corrective surgery (e.g., skin incision, repair of esophagus, trachea).
Abnormal Development of the Trachea - Tracheal Stenosis and Atresia
- Characterized by narrowing or blockage/obstruction of the trachea, occurring rarely and is associated with TEF.
- Unequal division of the foregut causes uneven partitioning impacting the esophagus and trachea.
- Treatment involves resection of the constricted portion and anastomosis (connecting the normal wider parts).
Development of the Bronchi
- The bronchi originate as a budding off of the trachea.
- Primary bronchial buds divide into left and right main bronchi.
- The right main bronchus divides into three secondary bronchial buds (lobar bronchi).
- The left main bronchus divides into two secondary bronchial buds (lobar bronchi).
- The secondary and tertiary bronchial buds divide into tertiary bronchi (segmental bronchi).
- The buds evolve into the future bronchopulmonary segments.
- The developing lungs expand into the pericardioperitoneal canals.
Development of the Bronchi - Bronchial Atresia
- Two types: Proximal (affecting lobar bronchus) and Peripheral (affecting segmental bronchi).
- Possible etiology: intrauterine ischemia commencing after the 16th week of gestation.
- Symptoms: Most cases are asymptomatic. Possible issues include recurrent infections, dyspnea (difficulty breathing), coughing, and wheezing. Diagnosing this will need a computed tomography scan or bronchofibroscopy.
- Treatment: Thoracoscopic surgery, localized resection, and/or lobectomy/segmentectomy.
Development of the Lungs
- The lungs progress through embryonic, pseudoglandular, canalicular, and saccular stages to the alveolar stage.
- The embryonic stage (weeks 4-7) features lung bud appearance.
- The pseudoglandular stage (weeks 5-17) is characterized by bronchial tree development and divisions.
- The canalicular stage (weeks 16-26) exhibits the formation of respiratory bronchioles, alveolar ducts, and primitive alveoli.
- The saccular stage (weeks 24 until birth) involves the development of terminal sacs (primitive alveoli). Type I pneumocytes form during this stage.
- The alveolar stage (8 months and beyond) occurs after birth and continues into childhood. Further maturation of the alveoli and establishment of the blood-air barrier occur. Surfactant production is crucial.
- Factors essential for the correct lung development in the fetus include adequate thoracic space, sufficient amniotic fluid volumes, and fetal breathing. At birth, fluid is removed from the lungs via various routes like the mouth, nose, lymphatic system, or through the blood vessels.
- Disorders like pulmonary agenesis, aplasia, or hypoplasia, are all potential issues in lung development which may result from vascular origins or disruptions in the blood flow during the early stages of gestation causing abnormalities.
- Another developmental abnormality is surfactant deficiency lung disease (neonatal respiratory distress syndrome). It's a common cause for respiratory distress in premature infants due to inadequate surfactant production.
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