Congenital Arrhinia and Pharyngeal Arches Quiz

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Questions and Answers

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?

  • Proboscis lateralis
  • Nasal hypoplasia
  • Polyrhinia (correct)
  • 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?

<p>5-6 years old (D)</p> Signup and view all the answers

What is the primary treatment for a condition characterized by a rudimentary nasal structure off-center from the midline?

<p>Heminose reconstruction (B)</p> Signup and view all the answers

What is NOT involved in the components of the pharyngeal arches?

<p>Pharyngeal arch ligaments (A)</p> Signup and view all the answers

What does the first pharyngeal cleft become in development?

<p>Acoustic meatus (A)</p> Signup and view all the answers

What is the primary function of pharyngeal arch 2?

<p>Facial expression and hyoid bone formation (C)</p> Signup and view all the answers

Which membrane separates the stomodeum from the primordial pharynx?

<p>Oropharyngeal membrane (C)</p> Signup and view all the answers

Which structure is formed from the first pharyngeal pouch?

<p>Middle ear cavity (A)</p> Signup and view all the answers

What is pulmonary agenesis characterized by?

<p>Complete absence of lung tissue (D)</p> Signup and view all the answers

What is the primary etiology associated with pulmonary aplasia?

<p>Developmental arrest of the primitive lung bud (D)</p> Signup and view all the answers

What is a common symptom of pulmonary hypoplasia?

<p>Recurrent pulmonary infections (D)</p> Signup and view all the answers

Which factor is NOT associated with neonatal respiratory distress syndrome?

<p>Infection during labor (D)</p> Signup and view all the answers

What treatment can enhance fetal lung maturation in cases of pulmonary hypoplasia?

<p>Antenatal corticosteroids (A)</p> Signup and view all the answers

Which symptom is common in neonates suffering from respiratory distress syndrome?

<p>Chest retractions (C)</p> Signup and view all the answers

Which of the following statements about surfactant is correct?

<p>Surfactant helps keep lungs fully expanded (A)</p> Signup and view all the answers

What is the expected outcome for most infants with mild symptoms of neonatal respiratory distress syndrome?

<p>They typically improve after 3-4 days (D)</p> Signup and view all the answers

What condition is characterized by an undersized lower jaw due to abnormal development of the first pharyngeal pouch?

<p>Micrognathia (C)</p> Signup and view all the answers

Which genetic abnormality is associated with DiGeorge syndrome?

<p>Microdeletion of chromosome 22 (D)</p> Signup and view all the answers

What treatment is NOT typically recommended for DiGeorge syndrome?

<p>Chemotherapy for autoimmunity (C)</p> Signup and view all the answers

What symptoms are included in the CATCH acronym for DiGeorge syndrome?

<p>Cardiac anomalies and cleft palate (B)</p> Signup and view all the answers

Which treatment is most crucial for resolving feeding problems associated with cleft palate in DiGeorge syndrome?

<p>Surgery to repair cleft palate (B)</p> Signup and view all the answers

What is a primary function of the larynx?

<p>Preventing food from entering the trachea (D)</p> Signup and view all the answers

How does the structure of the laryngeal orifice change during development?

<p>Changes from a sagittal slit to a T-shaped opening (D)</p> Signup and view all the answers

Which cartilage encircles the trachea completely?

<p>Cricoid cartilage (B)</p> Signup and view all the answers

What primarily induces the formation of the laryngeal ventricles?

<p>Recanalisation of the larynx (D)</p> Signup and view all the answers

What is the major developmental origin of the laryngeal epithelium?

<p>Endodermal origin (D)</p> Signup and view all the answers

What is the most common anomaly of the lower respiratory tract?

<p>Tracheoesophageal fistula (B)</p> Signup and view all the answers

Which of the following is a potential complication of tracheoesophageal fistula?

<p>Pneumonia (D)</p> Signup and view all the answers

What is a common symptom of tracheoesophageal fistula?

<p>Accumulation of liquid in the mouth (C)</p> Signup and view all the answers

What is the primary method of treatment for tracheoesophageal fistula?

<p>Corrective surgery (D)</p> Signup and view all the answers

What is a cause of tracheoesophageal fistula?

<p>Incomplete fusion of the esophageal folds (C)</p> Signup and view all the answers

What defines tracheal stenosis?

<p>A narrowing of the trachea (C)</p> Signup and view all the answers

How frequently does tracheoesophageal fistula occur in live births?

<p>1 in 3000 to 1 in 4500 (C)</p> Signup and view all the answers

What can result from the reflux of gastric contents through the fistula?

<p>Pneumonia or pneumonitis (A)</p> Signup and view all the answers

What is the relationship between tracheoesophageal fistula and esophageal atresia?

<p>Esophageal atresia commonly occurs with tracheoesophageal fistula. (D)</p> Signup and view all the answers

What results from unequal partitioning of the foregut?

<p>Narrowing of the trachea (tracheal stenosis) (B)</p> Signup and view all the answers

What primary function does the respiratory system NOT perform?

<p>Nutrient absorption (A)</p> Signup and view all the answers

During which week of embryonic development does the respiratory system begin to form?

<p>Week 4 (D)</p> Signup and view all the answers

The laryngotracheal groove is primarily a precursor to which of the following structures?

<p>Respiratory diverticulum (C)</p> Signup and view all the answers

What induces the bud formation and lung growth within the respiratory diverticulum?

<p>Retinoic acid from the mesoderm (C)</p> Signup and view all the answers

What type of tissue primarily gives rise to the connective tissue and cartilage of the lungs?

<p>Mesoderm (A)</p> Signup and view all the answers

Which cavity forms as the nasal pits deepen during development?

<p>Primordial nasal sacs (C)</p> Signup and view all the answers

What structure separates the nasal sacs from the oral cavity during development?

<p>Oronasal membrane (B)</p> Signup and view all the answers

What results from the apoptosis of an epithelial plug during nasal development?

<p>Nasal vestibule (C)</p> Signup and view all the answers

The primordial choanae are located at the junction between which two structures?

<p>Nasal cavity and pharynx (A)</p> Signup and view all the answers

The nasal placodes are considered to be which type of embryonic structure?

<p>Platelike thickening (B)</p> Signup and view all the answers

Which embryonic layer gives rise to the pulmonary epithelium and glands of the respiratory system?

<p>Endoderm (C)</p> Signup and view all the answers

What embryonic structure is described as an outpouching of the foregut?

<p>Respiratory diverticulum (B)</p> Signup and view all the answers

Which embryonic structure develops from the maxillary prominences?

<p>Upper jaw (C)</p> Signup and view all the answers

What is the incorrect association regarding the origins of various respiratory organs?

<p>Lungs - Ectoderm (B)</p> Signup and view all the answers

What is bronchial atresia characterized by?

<p>Focal interruption of bronchi (B)</p> Signup and view all the answers

What is the primary cause of bronchial atresia?

<p>Intrauterine ischemia after the 16th week of gestation (B)</p> Signup and view all the answers

Which diagnostic tools are used to identify bronchial atresia?

<p>Computed tomography scan and bronchofibroscopy (D)</p> Signup and view all the answers

What are possible symptoms of bronchial atresia?

<p>Coughing and wheezing (A)</p> Signup and view all the answers

What complication can arise due to bronchial atresia?

<p>Peripheral mucus impaction (D)</p> Signup and view all the answers

Which stage of lung development involves the formation of terminal sacs?

<p>Saccular stage (B)</p> Signup and view all the answers

At what stage does respiration become possible for fetuses?

<p>Canalicular stage (B)</p> Signup and view all the answers

What type of bronchi is referred to as tertiary bronchi?

<p>Segmental bronchi (C)</p> Signup and view all the answers

What is a primary function of type II pneumocytes?

<p>Secretion of pulmonary surfactant (C)</p> Signup and view all the answers

Which developmental stage has no respiratory bronchioles or alveoli present?

<p>Pseudoglandular stage (B)</p> Signup and view all the answers

When does the production of surfactant begin to occur?

<p>Weeks 20-22 of gestation (C)</p> Signup and view all the answers

What are the factors essential for normal lung development?

<p>Fetal breathing movements (B)</p> Signup and view all the answers

How is fluid cleared from the lungs at birth?

<p>Through mouth and nose during delivery (A)</p> Signup and view all the answers

During which stage do the terminal sacs form and establish gas-exchanging capabilities?

<p>Saccular stage (D)</p> Signup and view all the answers

What is a common treatment for polyrhinia?

<p>Surgical excision of the medial part of each nose (D)</p> Signup and view all the answers

Which abnormality is characterized by a rudimentary nasal structure positioned off-center?

<p>Proboscis lateralis (D)</p> Signup and view all the answers

Which of the following is a proposed etiology for congenital arrhinia?

<p>Failure of nasal epithelial plug resorption (C)</p> Signup and view all the answers

At what age is reconstructive surgery typically recommended for congenital arrhinia?

<p>5-6 years old (A)</p> Signup and view all the answers

What primary structure does the first pharyngeal pouch develop into?

<p>Middle ear cavity (A), Auditory tube (C)</p> Signup and view all the answers

What is the role of the pharyngeal arches during embryonic development?

<p>Support the lateral walls of the primordial pharynx (B)</p> Signup and view all the answers

How many pharyngeal arches are involved in the development of the human face and neck?

<p>Six (D)</p> Signup and view all the answers

What is one of the primary functions of the second pharyngeal arch?

<p>Facial expression (B)</p> Signup and view all the answers

What distinguishes congenital arrhinia from other nasal congenital abnormalities?

<p>Absence of nasal structures (D)</p> Signup and view all the answers

What characterizes pulmonary agenesis?

<p>Complete absence of lung tissue (A)</p> Signup and view all the answers

Which of the following complications is associated with pulmonary hypoplasia?

<p>Severe respiratory distress (A)</p> Signup and view all the answers

What is a common etiology associated with pulmonary aplasia?

<p>Congenital diaphragmatic hernia (C)</p> Signup and view all the answers

Which symptom is associated with neonatal respiratory distress syndrome?

<p>Cyanosis (D)</p> Signup and view all the answers

What treatment is typically used for improving fetal lung maturity?

<p>Corticosteroids (A)</p> Signup and view all the answers

What is a major symptom of impaired surfactant production in newborns?

<p>Grunting with breaths (B)</p> Signup and view all the answers

What is the expected outcome for most infants with neonatal respiratory distress syndrome?

<p>Over 90% survival with proper intervention (A)</p> Signup and view all the answers

What changes occur in the lungs due to prolonged intrauterine asphyxia in newborns?

<p>Irreversible changes to type II alveolar cells (A)</p> Signup and view all the answers

What is micrognathia primarily characterized by?

<p>Undersized lower jaw (D)</p> Signup and view all the answers

Which syndrome is associated with a microdeletion at chromosome 22q11.2?

<p>DiGeorge syndrome (A)</p> Signup and view all the answers

Which treatment is NOT commonly used for managing DiGeorge syndrome?

<p>Orthodontic braces (A)</p> Signup and view all the answers

What does the CATCH acronym stand for in relation to DiGeorge syndrome symptoms?

<p>Cardiac anomalies, Thymic hypoplasia, Cleft palate, Hypocalcemia (D)</p> Signup and view all the answers

What is the primary role of the larynx in the respiratory system?

<p>Preventing food from entering the lungs (A)</p> Signup and view all the answers

What developmental event changes the appearance of the laryngeal orifice from a sagittal slit to a T-shaped opening?

<p>Growth of arytenoid swellings (B)</p> Signup and view all the answers

Which cartilage structure is responsible for fully encircling the trachea?

<p>Cricoid cartilage (A)</p> Signup and view all the answers

Which complication is NOT typically associated with DiGeorge syndrome?

<p>Dental malocclusion (B)</p> Signup and view all the answers

What initiates the laryngeal ventricles' formation during development?

<p>Temporary occlusion of the laryngeal lumen (C)</p> Signup and view all the answers

What is the most common variety of tracheoesophageal fistula?

<p>Blind ending of the superior part of the esophagus and joining of the inferior part to the trachea (A)</p> Signup and view all the answers

What is an essential outcome of the rapid proliferation of epithelial tissue in the larynx development?

<p>Development of vocal cords (A)</p> Signup and view all the answers

What is a primary symptom of tracheoesophageal fistula in infants?

<p>Frequent coughing and choking when swallowing (C)</p> Signup and view all the answers

Which condition is associated with tracheoesophageal fistula?

<p>Esophageal atresia (B)</p> Signup and view all the answers

What is the recommended treatment for tracheoesophageal fistula?

<p>Corrective surgery (B)</p> Signup and view all the answers

Which of the following best describes a tracheoesophageal fistula?

<p>An abnormal opening causing reflux of stomach contents (A)</p> Signup and view all the answers

What causes the development of tracheoesophageal fistula?

<p>Incomplete division of the foregut into esophageal and respiratory parts (B)</p> Signup and view all the answers

What can excessive liquid accumulation in the mouth and upper respiratory tract indicate?

<p>Tracheoesophageal fistula (A)</p> Signup and view all the answers

What type of condition does tracheal stenosis represent?

<p>Narrowing of the trachea (D)</p> Signup and view all the answers

How often does tracheoesophageal fistula occur in live births?

<p>1 in 3000 to 1 in 4500 (B)</p> Signup and view all the answers

What is a potential consequence of gastric contents refluxing into the trachea through a fistula?

<p>Pneumonia or pneumonitis (C)</p> Signup and view all the answers

What is the primary role of the respiratory diverticulum during embryonic development?

<p>Development of the respiratory system (D)</p> Signup and view all the answers

Which embryonic layer contributes to the pulmonary epithelium and glands of the respiratory system?

<p>Endoderm (C)</p> Signup and view all the answers

What occurs during the 6th week of nasal cavity development?

<p>The oronasal membrane ruptures (C)</p> Signup and view all the answers

Which structure forms as an outpouching of the hollow foregut?

<p>Respiratory diverticulum (C)</p> Signup and view all the answers

What anatomical structures are primarily formed from the splanchnic mesoderm?

<p>Connective tissue and cartilage (D)</p> Signup and view all the answers

Which transcription factor is upregulated by retinoic acid during lung development?

<p>Tbx4 (C)</p> Signup and view all the answers

What occurs at the end of week 4 in the development of the respiratory system?

<p>Evagination of the laryngotracheal groove (A)</p> Signup and view all the answers

Which structure develops from the nasal placodes during embryogenesis?

<p>Nasal pit (C)</p> Signup and view all the answers

What predominantly influences bud formation and lung growth in the respiratory diverticulum?

<p>Chemical signaling from mesoderm (B)</p> Signup and view all the answers

Which anatomical feature is formed from the apoptosis of an epithelial plug during nasal development?

<p>Nasal vestibule (D)</p> Signup and view all the answers

Which is a function of the respiratory system?

<p>Sound and speech production (D)</p> Signup and view all the answers

What does the laryngotracheal groove give rise to in embryonic development?

<p>Tracheobronchial tree (B)</p> Signup and view all the answers

What defines the nasal vestibule's formation during development?

<p>Apoptosis of an epithelial plug (C)</p> Signup and view all the answers

What is the expected outcome of the primordial nasal sacs growing into the space underneath the forebrain?

<p>Deepening of nasal pits (B)</p> Signup and view all the answers

What is the primary symptom associated with bronchial atresia?

<p>Recurrent infections (B)</p> Signup and view all the answers

Which type of bronchial atresia involves a focal interruption of a segmental or subsegmental bronchus?

<p>Peripheral (D)</p> Signup and view all the answers

What can follow bronchial atresia due to obstruction in a lung segment?

<p>Hyperinflation of the obstructed lung segment (D)</p> Signup and view all the answers

What imaging technique is typically used for diagnosing bronchial atresia?

<p>Computed tomography scan (B)</p> Signup and view all the answers

What treatment is recommended for bronchial atresia?

<p>Thoracoscopic surgery (A)</p> Signup and view all the answers

What stage of lung development occurs between weeks 16-26 of gestation?

<p>Canalicular stage (C)</p> Signup and view all the answers

During which stage of lung development are terminal sacs (primitive alveoli) formed?

<p>Saccular stage (B)</p> Signup and view all the answers

Which type of bronchi is classified as tertiary bronchi?

<p>Segmental bronchi (D)</p> Signup and view all the answers

What physiological change is necessary for successful respiration after birth?

<p>Replacement of lung fluid with air (C)</p> Signup and view all the answers

Which type of bronchial niche complicates bronchial atresia due to mucus buildup?

<p>Bronchocele or mucocele (D)</p> Signup and view all the answers

What developmental factor is essential for normal lung growth?

<p>Adequate thoracic space (D)</p> Signup and view all the answers

At what gestational weeks does the respiratory diverticulum begin its development?

<p>Weeks 4-7 (B)</p> Signup and view all the answers

When does the production of pulmonary surfactant begin?

<p>20-22 weeks gestation (C)</p> Signup and view all the answers

Which structure forms from the differentiation of terminal sacs during lung development?

<p>Mature alveoli (B)</p> Signup and view all the answers

Flashcards

Respiratory system development

The process of forming the parts of the respiratory system, beginning in week 4 of development.

Laryngotracheal groove

An indentation in the floor of the pharynx that gives rise to parts of the respiratory system.

Respiratory diverticulum

A pouch-like outgrowth from the laryngotracheal groove.

Endoderm origin

The lining of the lung passages (pulmonary epithelium, glands, larynx, trachea, and bronchi) develop from the endoderm.

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Mesoderm origin

The surrounding tissues of the lungs (connective tissue, cartilage, and smooth muscle) develop from the splanchnic mesoderm.

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Nasal placodes

Thickened areas of ectoderm that develop into the nasal pits.

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Nasal Pits

Indentations in the embryo that develop into the nasal cavities.

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Oronasal membrane

Membrane separating the nasal sacs from the oral cavity.

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Nasal vestibule

Entrance to the nasal cavity.

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Primordial choanae

Initial openings between the nasal cavity and pharynx.

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Olfactory epithelium

Specialized epithelial cells that are sensitive to smells.

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Olfactory receptor cells

Specialized cells in the nose that detect odors.

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Facial prominences

Five embryonic structures that give rise to parts of the face.

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Frontonasal prominence

Embryonic structure giving rise to parts of the forehead, nose and bridge.

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Tbx4

Transcription factor that induces lung growth and differentiation.

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Congenital arrhinia

Absence of external and internal nasal structures.

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Polyrhinia

Complete duplication of the nose, forming two noses.

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Proboscis lateralis

Rudimentary nasal structure or appendage off-centre from face's midline.

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Pharyngeal arches

Support lateral walls of the primordial pharynx, formed from mesenchyme.

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Pharyngeal pouches

Internal sacs derived from endoderm, contributing to various head/neck structures.

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Pharyngeal clefts

External grooves that separate the pharyngeal arches, develop into ear canal.

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Pharyngeal membranes

Sites where pharyngeal clefts & pouches meet, form tympanic membrane.

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Pharyngeal arch artery

Blood vessels within pharyngeal arches, important for development.

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Pharyngeal arch cartilage

Cartilage within pharyngeal arches, forming structures like hyoid bone.

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Pharyngeal arch muscles

Muscles developing within pharyngeal arches, associated with chewing etc.

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Micrognathia

Undersized lower jaw, an abnormal development of the pharynx.

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Pierre Robin syndrome

A condition causing micrognathia, among other issues.

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DiGeorge syndrome

A genetic disorder causing abnormal development in throat and other systems.

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22q11.2 deletion

A missing piece on chromosome 22, a key component in DiGeorge syndrome.

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Laryngeal orifice

Opening between the pharynx & larynx which allows air to enter the trachea.

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Laryngeal lumen

The hollow space inside the larynx.

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Vocal folds

Folds within the larynx, crucial for creating sounds.

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Thyroid cartilage

Cartilage protecting and supporting vocal cords in the larynx.

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Tracheoesophageal fistula

An abnormal connection between the trachea and esophagus, usually occurring during fetal development.

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Esophageal atresia

A condition where the esophagus is completely or partially blocked, often associated with tracheoesophageal fistula.

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What causes tracheoesophageal fistula?

Incomplete division of the foregut into esophageal and respiratory parts, incomplete fusion of the esophageal folds, or defective tracheoesophageal septum.

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How does tracheoesophageal fistula affect swallowing?

Fluid accumulates in the mouth and upper respiratory tract, leading to coughing and choking during swallowing. Contents may reflux from the stomach into the lungs.

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Bifurcation

The point where something divides into two branches, like the trachea branching into the bronchi.

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Tracheal stenosis

Narrowing of the trachea, making breathing difficult.

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Tracheal atresia

Complete blockage of the trachea, preventing air from passing.

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How are tracheal stenosis and atresia treated?

Resection and anastomosis, removing the narrowed portion and reconnecting the normal wider portions.

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Primary bronchial buds

Early branches of the bronchi that develop from two lateral outpocketings during the fifth week of development.

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Secondary and tertiary bronchial buds

Further branching of the bronchi, forming the secondary bronchi (right - 3, left - 2) and tertiary bronchi (segmental bronchi) during lung development.

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Pulmonary agenesis

Complete absence of lung tissue, usually affecting one side (unilateral), caused by disruption of blood flow during early fetal development.

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Pulmonary aplasia

Complete developmental arrest of the lung bud, meaning the lung never started to form properly.

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Pulmonary hypoplasia

Small, underdeveloped lungs, affecting either one or both sides (unilateral or bilateral), caused by various factors like congenital diaphragmatic hernia or changes in growth factors.

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Neonatal respiratory distress syndrome (NRDS)

Breathing difficulty in newborns, caused by a lack of surfactant, often due to premature birth or intrauterine asphyxia.

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Surfactant

A substance that lines the lungs helping them stay open and allow the newborn to breathe. It's like a 'soap' that helps the lungs expand.

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What causes NRDS?

Premature birth and prolonged intrauterine asphyxia are the main causes. Both lead to surfactant insufficiency - the substance that keeps the lungs expanded.

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NRDS Symptoms

Fast breathing, grunting sound with each breath, changes in lip/finger/toe color, widened nostrils with breathing, and chest retractions.

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NRDS Treatment

Prevention with corticosteroids before delivery, supplementary oxygen, and artificial surfactant are key elements. For cases with mild symptoms, the baby usually improves within a few days.

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Bronchial Atresia

A condition where a bronchus fails to develop completely, leading to a blockage in the airway.

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Proximal Bronchial Atresia

A blockage in a lobar bronchus, affecting a larger area of the lung.

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Peripheral Bronchial Atresia

A blockage in a segmental or subsegmental bronchus, affecting a smaller area of the lung.

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Etiology of Bronchial Atresia

Often caused by intrauterine ischemia (lack of blood flow) after the 16th week of gestation.

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Symptoms of Bronchial Atresia

Most individuals are asymptomatic, but some may experience recurrent infections, dyspnea (difficulty breathing), coughing, or wheezing.

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Complications of Bronchial Atresia

Can lead to mucus build-up (bronchocele or mucocele), hyperinflation (overexpansion) of the affected lung segment.

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Diagnosis of Bronchial Atresia

Usually diagnosed using a computed tomography (CT) scan or bronchofibroscopy.

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Treatment for Bronchial Atresia

Can be treated with thoracoscopic surgery (keyhole surgery), local resection (removing a small part), or lobectomy/segmentectomy (removing a larger part of the lung).

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Embryonic Stage of Lung Development

From week 4-7, lung buds form, followed by primary bronchial buds, main bronchi, secondary bronchi, and tertiary bronchi.

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Pseudoglandular Stage of Lung Development

From week 5-17, the bronchial tree develops, with tertiary bronchi branching into primary bronchioles and then terminal bronchioles, but no respiratory bronchioles or alveoli are present, making respiration impossible.

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Canalicular Stage of Lung Development

From week 16-26, terminal bronchioles divide into respiratory bronchioles, which branch into alveolar ducts, and the mesenchyme becomes highly vascular, making respiration possible.

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Saccular Stage of Lung Development

From 24 weeks to birth, terminal sacs (primitive alveoli) form, capillaries establish close contact for gas exchange, and blood-air barrier forms, enabling survival.

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Alveolar Stage of Lung Development

From 8 months to childhood, terminal sacs differentiate into mature alveoli, with well-developed epithelial endothelial contacts, allowing for efficient gas exchange, and alveoli continue to form throughout early adulthood.

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Type I Pneumocytes

Squamous epithelial cells in the alveoli that are responsible for gas exchange.

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Type II Pneumocytes

Rounded secretory epithelial cells in the alveoli that produce pulmonary surfactant, which helps keep the alveoli open.

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What is the main function of the respiratory system?

The main function of the respiratory system is gas exchange, which allows for the uptake of oxygen and the release of carbon dioxide. It also plays a role in smell, filtration, sound production, and waste elimination.

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What germ layer forms the pulmonary epithelium and glands?

The endoderm, a primary germ layer, develops into the lining of the respiratory system, including the pulmonary epithelium and glands.

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What germ layer forms the cartilage and smooth muscle of the respiratory system?

The splanchnic mesoderm, a specific type of mesoderm, gives rise to the connective tissue, cartilage, and smooth muscle of the respiratory system.

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What is the respiratory diverticulum?

The respiratory diverticulum is an outpouching from the ventral wall of the foregut that develops into the respiratory system.

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What is the role of retinoic acid in lung development?

Retinoic acid, produced by the mesoderm, upregulates the transcription factor Tbx4 in the endoderm near the respiratory diverticulum, inducing lung bud formation and growth.

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What are the five facial primordia?

The five facial primordia are embryonic structures that develop into features of the face: Frontonasal prominence, maxillary prominences (2), and mandibular prominences (2).

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What are nasal placodes?

Nasal placodes are thickened areas of ectoderm that develop into the nasal pits, the precursors of the nasal cavities.

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How does the nasal cavity develop?

Nasal placodes become depressed to form pits. The surrounding mesenchyme proliferates, forming prominences. Nasal pits deepen and form sacs. These sacs grow dorsally, forming the nasal cavity.

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What happens when the oronasal membrane ruptures?

The rupture of the oronasal membrane allows communication between the nasal and oral cavities, forming the primordial choanae.

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What is the role of the olfactory epithelium?

The olfactory epithelium contains specialized olfactory receptor cells, which detect odors and whose axons form the olfactory nerves, connecting to the brain.

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What is the effect of the development and apoptosis of an epithelial plug?

The development and apoptosis of an epithelial plug forms the nasal vestibule, the entrance to the nasal cavity.

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What are the potential developmental issues with the nasal cavity?

Congenital arrhinia (absence of nose), polyrhinia (two noses), and proboscis lateralis (rudmentary nasal appendage) are some developmental anomalies of the nasal cavity.

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What is the pharynx formed from?

The pharynx, a part of the throat, develops from the primordial pharynx, which is formed from mesenchyme and endoderm.

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What are potential defects in the development of the pharynx?

Micrognathia (small lower jaw) and Pierre Robin syndrome (including micrognathia) can result from abnormal pharyngeal development.

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What is the laryngeal orifice?

The laryngeal orifice is the opening between the pharynx and larynx, allowing air passage to the trachea.

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What does each pharyngeal arch contain?

Each pharyngeal arch contains a unique set of structures: a pharyngeal arch artery (blood vessel), pharyngeal arch cartilage (cartilage), pharyngeal arch muscles (muscles), and pharyngeal arch nerves (nerves).

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What are the six pharyngeal arches?

The six pharyngeal arches are numbered 1 to 6. Each arch has a specific role in development. Arch 1 contributes to chewing, arch 2 to smiling, arches 3 and 4 to swallowing, arch 5 has limited contributions, and arch 6 is involved with speaking.

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Pharyngeal Derivatives

Pharyngeal pouches give rise to various structures in the head and neck. The first pouch contributes to the middle ear cavity and auditory tube, the second contributes to the palatine tonsil, the third forms the inferior parathyroid glands and the thymus, and the fourth and fifth form the posterior parathyroid glands and the ultimobranchial body.

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What is micrognathia?

An abnormal development of the pharynx resulting in an undersized lower jaw, also known as mandibular hypoplasia.

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What causes micrognathia?

Micrognathia can be caused by conditions like Pierre Robin syndrome, Trisomy 13 (Patau syndrome), and Fetal alcohol syndrome.

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What happens during a Laryngeal Orifice Transformation?

The laryngeal orifice changes from a sagittal slit to a T-shaped opening due to the growth of arytenoid swellings towards the tongue.

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What is the role of the thyroid cartilage?

The thyroid cartilage protects and supports the vocal cords within the larynx.

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What are the functions of the larynx?

The larynx plays crucial roles in breathing, producing vocal sounds, and preventing food from entering the trachea and lungs.

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What are the origins of the larynx?

The larynx's epithelial lining originates from the endoderm, while its cartilages and muscles develop from mesenchyme in the 4th and 6th pharyngeal arches.

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How are the vocal folds formed?

The vocal folds are formed by folds of mucous membrane within the larynx that develop from the laryngeal ventricles.

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What is the purpose of the epiglottis?

The epiglottis is responsible for preventing food and drink from entering the respiratory tract during swallowing.

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Most common anomaly of the lower respiratory tract

Tracheoesophageal fistula is the most frequent abnormality of the lower part of the breathing system, affecting about 1 in 3000 to 4500 newborns.

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Symptoms of tracheoesophageal fistula

Babies with this condition may have excessive saliva, choking when feeding, and regurgitation (spitting up). They may also cough and have trouble breathing due to gastric contents entering the lungs.

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Treatment for tracheoesophageal fistula

Surgery is typically required to repair the abnormal connection and restore normal flow of food and air.

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Treatment for tracheal stenosis

Surgery to widen the trachea and remove the narrowed portion is the primary treatment for tracheal stenosis.

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Secondary bronchi

These are the branches that directly branch off from the main bronchi. The right bronchus forms three secondary bronchi, while the left forms two.

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Tertiary bronchial buds

These buds give rise to the segmental bronchi, which further divide the airways into the future bronchopulmonary segments. There are ten in the right lung and eight in the left.

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Bronchial Atresia Etiology

Often caused by lack of blood flow (ischemia) in the womb after week 16 of pregnancy.

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Bronchial Atresia Symptoms

Usually no symptoms, but sometimes recurrent infections, breathing difficulties (dyspnea), coughing, or wheezing.

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Bronchial Atresia Complications

Can lead to mucus build-up (bronchocele or mucocele) and overexpansion (hyperinflation) of the affected lung part.

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Bronchial Atresia Diagnosis

Usually diagnosed by a CT scan (detailed image) or bronchofibroscopy (looking into the airway).

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Bronchial Atresia Treatment

Can be treated by surgery, such as thoracoscopic (small incisions), local resection (removing a small part), or lobectomy/segmentectomy (removing a larger part of the lung).

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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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