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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4534_Библиотеки_им_академика_М_И_Перельмана.pdf
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- •Contents
- •1.1.9 Laryngeal Atresia
- •1.1.10 Laryngeal Webs
- •1.1.11 Congenital Subglottic Stenosis
- •1.1.12 Laryngeal Cleft
- •1.1.13 Tracheoesophageal Fistula
- •1.1.14 Tracheal Bronchus
- •1.2.2 Mesenchyme Development
- •1.2.4 Lung Development
- •1.2.4.1 Embryonic Stage
- •1.2.4.2 Pseudoglandular Stage
- •1.1.1 Oral Cavity
- •1.1.2 Nasal Cavity
- •1.1.3 Palate
- •1.1.4 Primitive Pharynx
- •1.1.5 Upper Airway Anomalies
- •1.1.6 Cleft Lip/Palate
- •1.1.7 Choanal Atresia
- •1.1.8 Laryngomalacia
- •1.2.4.3 Canalicular Stage
- •1.2.4.4 Saccular Stage
- •1.2.4.5 Alveolar Stage
- •1.2.5 Congenital Respiratory System Defects
- •1.2.5.1 Tracheal Agenesis
- •1.2.5.2 Congenital Tracheal Stenosis
- •1.2.5.3 Lung Agenesis
- •1.2.5.4 Lung Hypoplasia
- •References
- •2.1 Introduction
- •2.2 Nasal Cavity
- •2.2.1 Vestibule
- •2.2.2 Respiratory Mucosa
- •2.2.3 Olfactory Mucosa
- •Supporting Cells
- •Basal Cell
- •Olfactory Receptor Cell (Bipolar Neuron)
- •Brush Cell (Microvillar Cell)
- •2.2.3.2 The Lamina Propria
- •2.2.3.3 Olfactory Glands (Bowman’s Glands)
- •2.3 Paranasal Sinuses
- •2.4 Pharynx
- •2.5 Larynx
- •2.6 Trachea
- •2.6.1.1 Ciliated Columnar Cells
- •2.6.1.2 Goblet Cells
- •2.6.1.3 Brush Cells
- •2.6.1.4 Basal Cells
- •2.6.1.5 Enteroendocrine System Cells (Kulchitsky Cells or DNES Cells)
- •2.6.2 Lamina Propria
- •2.7 Lungs
- •2.7.1 Pleura
- •2.7.2 Bronchi
- •2.7.3 Bronchioles
- •2.7.3.1 Terminal Bronchioles
- •2.7.3.2 Respiratory Bronchioles
- •2.7.4 Ductus Alveolaris
- •2.7.5 Alveoli
- •2.7.5.2 Type II Alveolar Cell (Septal Cell, Large Alveolar Cell)
- •References
- •3.1.1.3 Nerves
- •Ophthalmic Division
- •Maxillary Division
- •Parasympathetic Nerve Supply
- •3.1.1.4 Bony Anatomy
- •3.1.1.5 Cartilaginous Pyramid
- •3.1.1.6 Structure
- •External Nasal Anatomy
- •Internal Nasal Anatomy
- •3.1.2 Nasal Physiology
- •3.1.2.1 Nasal Airflow
- •3.1.2.2 Abnormal Nasal Physiology
- •3.2.1 Larynx Anatomy
- •Cricoid Cartilage
- •Thyroid Cartilage
- •Epiglottis
- •Arytenoid Cartilages
- •Corniculate Cartilages
- •Cuneiform Cartilages
- •Extrinsic Ligaments
- •Intrinsic Ligaments
- •Laryngeal Cavity
- •Piriform Recesses
- •Cricothyroid Muscles
- •Posterior Cricoarytenoid Muscles
- •Lateral Cricoarytenoid Muscles
- •Transverse Arytenoid Muscle
- •Thyroarytenoid Muscles
- •Superior Laryngeal Nerve
- •Arteries
- •Veins
- •Lymphatics
- •Swallowing
- •Respiration
- •Phonation
- •3.2.2.1 Reflex Glottic Closure
- •References
- •4.1 Introduction
- •4.2.1 Choanal Atresia
- •4.2.2 Pyriform Aperture Stenosis
- •4.2.3 Cleft Lip Nasal Deformity
- •4.2.4 Nasolacrimal Duct Cysts
- •4.2.5 Encephaloceles
- •4.3 Craniofacial Anomalies
- •4.3.1 Pierre Robin Syndrome
- •4.3.2 Treacher-Collins Syndrome
- •4.3.3 Crouzon Syndrome
- •4.3.4 Down Syndrome
- •4.3.5 Apert Syndrome
- •4.4.1 Thyroglossal Duct Cyst
- •4.4.2 Laryngomalacia
- •4.4.3 Vocal Fold Paralysis
- •4.4.5 Subglottic Stenosis
- •4.4.6 Subglottic Hemangioma
- •4.4.7 Laryngeal Cysts
- •4.4.8 Laryngeal Cleft
- •4.5 Conclusion
- •References
- •5.1 Innate Immunity
- •5.2 Adaptive Immunity
- •References
- •6.1 Introduction
- •6.2 Innate Immunity
- •6.3 Adaptive Immunity
- •References
- •7.1 Introduction
- •References
- •8: Respiratory Microbiome
- •8.1 Introduction
- •8.2.1 Childhood Asthma
- •8.2.2 Asthma Exacerbation
- •8.3 Bacteriome
- •8.4 Virome
- •8.5 Mycobiome
- •References
- •9.1 Introduction
- •References
- •10.1 Introduction
- •10.3.3 The Appointment Process, Explained
- •10.3.5 Parental Involvement
- •10.4 Coordinating Care When Your Child Is Ill
- •10.4.3 Exhibit Cohesion
- •10.6 Conclusion
- •References
- •11.1 Introduction
- •11.2 Nasal Cavity
- •11.2.1 Choanal Atresia
- •11.2.2 Rhinosinusitis
- •11.2.4 Juvenile Nasopharyngeal Angiofibroma
- •11.3 Pharynx
- •11.4 Nasopharynx
- •11.4.1 Adenoid Hypertrophy
- •11.4.2 Nasopharyngeal Carcinoma
- •11.5 Oropharynx
- •11.5.1 Thyroglossal Duct Cyst
- •11.6 Hypopharynx
- •11.6.1 Retropharyngeal Abscess
- •11.6.2 Lymphatic Malformation
- •11.6.4 Lymphoma
- •11.6.5 Rhabdomyosarcoma
- •11.7 Larynx
- •11.7.1 Subglottic Stenosis
- •11.7.2 Laryngotracheal Papillomatozis
- •11.7.3 Croup
- •11.7.4 Epiglottitis
- •11.7.5 Foreign Body Aspiration
- •References
- •12.2.1 Plain Radiography
- •12.2.1.1 The Thymus
- •Tracheal Buckling
- •Hilum
- •Diaphragm
- •Mediastinal Borders
- •Lung Opacities
- •Cystic Lung Diseases
- •Pulmonary İnterstitial Emphysema (PIE)
- •Unilateral Hyperlucent Lung
- •12.2.2 Fluoroscopy
- •12.2.3 Ultrasound
- •12.2.4 Computed Tomography
- •12.2.5 Magnetic Resonance Imaging (MRI)
- •12.2.6 Angiography
- •12.2.7 Positron Emission Tomography (PET)
- •12.3 Conclusion
- •References
- •13.1 Introduction
- •13.2 Nasal Diagnostic Procedures
- •13.2.1 Indications
- •13.2.2 Contraindications
- •13.2.3 Anatomical Features
- •13.2.4 Technical Considerations
- •13.2.5 Technique
- •13.2.5.1 First Pass
- •13.2.5.2 Second Pass
- •13.2.5.3 Third Pass
- •13.3 Flexible Laryngoscopy
- •13.4 Direct Laryngoscopy
- •13.5 Video Laryngoscopy
- •13.5.1 Indications
- •13.5.2 Contraindications
- •13.5.3 Outcomes
- •13.5.4 Equipment
- •13.5.5 Approach Considerations
- •References
- •14.1 Upper Airways
- •14.2.3 Laryngeal Pathologıes
- •References
- •15.1 Introduction
- •15.2 Airway Measurements
- •References
- •16.1 Introduction
- •16.2 Background
- •References
- •17: Allergen Testing: Purpose, Procedure, Interpretation
- •17.1 Introduction
- •17.2 Tests
- •17.2.1 Skin Tests
- •17.2.3 Component Resolved Diagnosis (CRD)
- •17.2.4 Tryptase
- •17.2.5 Basophil Activation Test (BAT)
- •17.2.6 Provocation Tests
- •17.2.7 Nasal sIgE
- •17.2.8 Nasal Smear Eosinophilia
- •17.2.9 Eosinophilic Cationic Protein (ECP)
- •References
- •18: Smell Testing: Purpose, Procedure, Interpretation
- •18.1 Introduction
- •18.2 Possible Olfactory Disorder Diagnosis
- •18.2.1 Conductive Defects
- •18.2.3 Inherited Disorders
- •18.2.3.1 Hormonal Disturbances
- •18.4 Odor Threshold Tests
- •18.8.1 Butanol Threshold Test
- •18.8.1.1 The Penn State University Odor Identification Exam
- •18.8.2 Cross-Cultural Smell Identification Test
- •18.8.3 Sniffin’ Sticks
- •References
- •19: Taste Testing: Purpose, Procedure, Interpretation
- •19.1 Introduction
- •19.2 Definitions
- •19.2.1 Taste Dysfunction Abnormalities
- •19.4.1 Taste Dysfunction
- •19.4.2 COVID-19
- •19.5 Taste Disorder Diagnosis
- •19.6.2 Magnitude Matching
- •19.6.3 Spatial Test
- •References
- •20.1 Introduction
- •20.2 Primary Otalgia Causes
- •20.2.1 Auricle
- •20.2.1.1 Infections
- •20.2.1.2 Trauma
- •20.2.1.3 Allergic Angioedema
- •20.2.1.4 Thermal Damage
- •20.2.2 External Auditory Canal
- •20.2.2.1 Otitis Externa
- •20.2.2.2 Malignant Otitis Externa
- •20.2.2.3 Eczematous Dermatitis
- •20.2.2.4 Furunculosis
- •20.2.2.5 Foreign Body
- •20.2.2.6 Cerumen Impaction
- •20.2.2.7 Tumors
- •20.2.3 Middle Ear
- •20.2.3.1 Acute Otitis Media
- •20.2.3.3 Eustachian Tube Dysfunction
- •20.2.3.4 Cholesteatoma
- •20.2.3.5 Trauma
- •20.3 Secondary Otalgia Causes
- •20.3.1 Oropharyngeal Infections
- •20.3.2 Dental Causes
- •20.3.3 Auricular Lymphadenitis
- •20.3.4 Neck Abscess
- •20.3.5 Parotitis
- •20.3.6 Temporomandibular Joint Dysfunction
- •20.3.7 Sinusitis
- •20.4 Differential Diagnosis
- •References
- •21.1 Introduction
- •21.2 Bacterial Otitis Externa
- •21.3 Acute Otitis Media
- •21.4 Chronic Suppurative Otitis Media
- •21.5 Foreign Body
- •21.5.1 Cerumen
- •21.5.2 Tympanostomy Tube Drainage
- •21.5.3 Traumatic Cerebrospinal Fluid Otorrhea
- •21.5.5 Necrotizing Otitis Externa (Malignant External Otitis)
- •21.5.6 Neoplasms
- •21.5.7 Polyps
- •21.5.8 Otomycosis
- •21.5.9 First Branchial Cleft Cysts
- •21.5.10 Cholesteatoma
- •21.5.11 Spontaneous Cerebral Spinal Fluid Otorrhea
- •References
- •22.1 Introduction
- •22.4 Congenital Causes
- •22.4.1 Choanal Atresia
- •22.4.2 Pyriform Apertura Stenosis
- •22.4.3 Nasal Midline Congenital Masses
- •22.4.3.1 Nasal Dermoid Cyst
- •22.4.3.2 Nasal Glioma
- •22.4.3.3 Encephalocele (Encephalo-Meningocele)
- •Differential Diagnosis
- •22.4.3.4 Nasolacrimal Duct Cyst (Dacryocystocele)
- •22.5 Infectious Causes
- •22.5.1.1 Rhinitis Etiology
- •22.5.2 Neonatal Rhinitis
- •22.5.3 Bacterial or Viral Rhinitis
- •22.5.4 Iatrogenic Rhinitis
- •22.5.5 Infectious Rhinitis (Rhinosinusitis)
- •22.6 Adenoid Hypertrophy
- •22.7 Inflammatory Causes
- •22.7.1 Allergic Rhinitis
- •22.7.2 Nonallergic Rhinitis
- •22.7.3 Eosinophilic Nonallergic Rhinitis (NARES)
- •22.7.4 Nasal Polyp
- •22.7.5 Antrochoanal Polyp
- •22.7.6 Inferior Turbinate Hypertrophy
- •22.8 Neoplasia
- •22.8.1 Benign Tumors (Juvenile Nasopharyngeal Angiofibroma)
- •22.8.2 Malignant Tumors
- •22.9 Systemic Causes
- •22.9.1 Cystic Fibrosis
- •22.9.2 Primary Ciliary Dyskinesia
- •22.10 Trauma/Iatrogenic Causes
- •22.10.1 Nasal Trauma-Septal Hematoma
- •22.10.2 Septum Deviation
- •22.10.3 Nasal Foreign Bodies
- •References
- •23.1 Introduction
- •23.2 Pathophysiology
- •23.3 Allergic Rhinitis
- •23.4 Non-allergic Rhinitis
- •23.5 Infectious Rhinitis
- •23.6.1 Vasomotor Rhinitis
- •23.7 Evaluation
- •23.8 Diagnosis
- •23.9 Treatment
- •23.10 Prognosis
- •23.11 Conclusion
- •References
- •24.1 Introduction
- •24.2 Pathogenesis
- •24.3 Diagnosis
- •24.3.1 History
- •24.3.2 Examination
- •24.4 Differential Diagnoses
- •24.5 CSF Rhinorrhea
- •24.5.1 CSF Physiology
- •24.5.1.1 Pathogenesis
- •24.6 Diagnosis
- •24.6.1 Chemical Diagnosis
- •24.6.2 Imaging Diagnosis
- •24.7 Treatment
- •24.7.1 Surgical Technique
- •References
- •25.1 Introduction
- •25.1.1 Waldeyer Ring
- •25.3 Anatomy
- •25.3.1 Lymphatic Drainage
- •25.3.1.1 Nerve Supply
- •25.6 Tonsillary Hypertrophy
- •25.7 Physical Examination
- •25.8.1 Obstructive Sleep Apnea
- •References
- •26.1 Introduction
- •26.5 Halitosis Physiopathology
- •26.6.1 Oral Halitosis (Intraoral Halitosis, Oral Malodor)
- •26.6.1.1 Periodontal Infections
- •26.6.1.2 Tongue Oriented Halitosis
- •26.6.1.3 Peritonsillar Abscess
- •26.7 Paranasal Sinus Diseases
- •26.8 Adenoid Vegetation
- •26.9 Chronic Pharyngitis
- •26.10 Chronic Tonsillitis
- •26.11 Tonsillolith
- •26.12 Non-Oral Halitosis
- •26.13 Gastroesophageal Reflux
- •26.14 Diagnosis
- •26.14.1 Organoleptic Measurement
- •26.14.2 Sulfur Monitoring
- •26.14.2.1 Indirect Methods
- •26.14.2.3 Ammonia Monitoring
- •26.14.2.4 Polymerase Chain Reaction (PCR)
- •26.15 Physical Examination
- •References
- •27.1 Introduction
- •27.2 Epidemiology
- •27.4 Diagnosis
- •27.5.1 Clinical Assessment
- •27.6 Treatment
- •27.6.1 Voice Therapy
- •27.7 Phonosurgery
- •References
- •28.1 Introduction
- •28.2 Epidemiologic Characteristics
- •28.3 Swallowing Physiologic Phases
- •28.3.1.1 Prematurity
- •28.3.1.2 Neuromuscular
- •28.3.1.5 Cardiopulmonary Disease
- •28.4 Symptoms
- •28.5 Clinical Feeding Assessment
- •28.7 Flexible Endoscopic Swallowing Evaluation
- •28.8 Imaging
- •28.9 Endoscopic Assessments
- •28.9.1 High-Resolution Manometry
- •28.10 Medical Management
- •28.11 Surgical Management
- •28.11.1 Ankyloglossia
- •28.11.2 Laryngomalacia
- •28.11.3 Laryngeal Cleft
- •28.12 Conclusions
- •References
- •29.1 Introduction
- •29.2 Reactive Lymph Node Enlargements
- •29.3 Vaccines
- •29.4 Acute Suppurative Lymphadenitis
- •29.6 Granulomatous Lymphadenitis
- •29.6.1 Mycobacterial Infection
- •29.6.2 BCG Vaccine
- •29.6.3 Cat-Scratch Disease
- •29.6.4 Sarcoidosis
- •29.6.5 Kikuchi-Fujimoto Disease
- •29.7 Malignancies
- •29.8 Diagnosis
- •References
- •30.1 Introduction
- •30.2 Upper Airway Cough Syndrome
- •30.3 Chronic Rhinosinusitis
- •30.5 Otogenic Cough
- •30.6 Laryngeal Clefts
- •30.7 Conclusion
- •References
- •31.1 Introduction
- •31.5.1 Vocal Cord Disfunction (VCD)
- •31.5.2 Obstructive Sleep Apnea Syndrome (OSAS)
- •31.5.3 Allergic or Non-Allergic Rhinitis
- •31.6 Conclusion
- •References
- •32.1 Introduction
- •32.2.1 Non-massive Hemoptysis
- •32.2.2 Massive Hemoptysis
- •32.4 Diagnostic Evaluation
- •32.4.1 History
- •32.4.1.1 Infection Warning Signs
- •32.4.1.2 Choking
- •32.4.1.3 Exposures
- •32.4.1.4 Underlying Medical Problems
- •32.4.2 Physical Examination
- •32.4.3 Laboratory Evaluation
- •32.4.4 Imaging
- •32.5.1 Respiratory Illness
- •32.5.3 Trauma
- •32.5.4 Hemoptysis Mimics
- •References
- •33.1 Introduction
- •33.6 Conclusion
- •References
- •34: Pediatric Allergic Rhinitis: Otolaryngology Perspective
- •34.1 Introduction
- •34.2 Epidemiology
- •34.2.1 Prevalence
- •34.2.2 Risk factors
- •34.3.1 Classical Pathway
- •34.3.2 Nasal Pathway
- •34.4.2 Physical Examination
- •34.4.3 Diagnostic Tests
- •34.4.4 Nasal Cytology
- •34.4.5 Imaging
- •34.5.1 Adenoid Hypertrophy
- •34.5.2 Nasal Septal Deviation
- •34.5.3 Chronic Rhinosinusitis
- •34.5.4 Turbinate Hypertrophy
- •34.5.5 Nasal Foreign Body
- •34.5.6 Other Clinical Conditions
- •34.6.1 Saline Irrigation (Douching)
- •34.7 Treatment
- •34.7.1 Oral Antihistamines
- •34.7.2 Intranasal Steroids
- •34.7.3 Leukotriene Inhibitors
- •34.7.5 Oral Steroids
- •34.7.6 Intranasal Antihistamines
- •34.7.7 Immunotherapy (Sublingual-Subcutaneous)
- •34.8 Conclusion
- •References
- •35: Allergic Rhinitis: Pediatric Pulmonologist Perspective
- •35.1 Introduction
- •35.2.1 Epidemiological Relationship
- •35.2.4 Immunopathology
- •35.2.7 Non-pharmaceutical Treatment Method
- •35.2.8 Pharmaceutical Medication Policy
- •35.2.9 Immunotherapy Against Allergens
- •35.6 Conclusion
- •References
- •References
- •37.1 Introduction
- •37.2 Adenoid Hypertrophy
- •37.7 Preoperative Evaluation
- •37.8 Contraindications
- •37.9 Complications
- •37.9.1 Bleeding
- •37.9.2 Hypernasality
- •37.9.3 Surgical Traumas
- •37.9.4 Torticollis
- •37.9.5 Otitis Media
- •37.9.6 Psychological Trauma
- •37.9.7 Nasopharyngeal Stenosis
- •37.9.8 Recurrence
- •37.10 Postoperative Care
- •37.11 Surgery
- •37.12.1 Adenoiditis
- •References
- •38.1 Introduction
- •38.2 Anatomy
- •38.2.1 Palatine Tonsils (Faucial Tonsils)
- •38.2.2 Lingual Tonsil
- •38.2.3 Adenoids (Pharyngeal Tonsil)
- •38.2.4 Tubal Tonsils
- •38.5.1 Viral Tonsillitis
- •38.5.2 Bacterial Tonsillitis
- •38.5.3 Candida
- •38.6.1 Suppurative Complications
- •38.6.1.1 Peritonsillar Abscess (Quincy Tonsil)
- •Lemierre’s Syndrome
- •38.6.2 Nonsuppurative Complications
- •38.6.2.1 Acute Rheumatic Fever
- •38.6.2.2 Poststreptococcal Glomerulonephritis
- •38.6.2.3 Scarlet Fever
- •38.6.2.6 Palmoplantar Pustulosis (PPP)
- •38.6.2.7 IgA Nephropathy
- •38.7 Clinical Manifestation
- •38.7.1 Infection
- •38.7.2 Obstruction
- •38.7.3 Neoplasia
- •38.8 Diagnosis
- •38.8.2 Physical Examination
- •38.8.3 Laboratory
- •38.8.4 Imagining
- •38.8.5 Polysomnography
- •38.9 Treatments
- •38.9.1 Medical Treatment
- •38.9.2 Surgery
- •38.9.2.2 Tonsillectomy
- •38.9.3.1 Intraoperative Complications
- •38.9.3.4 Postoperative Long-Term Complications (>weeks)

62
E. K. Yardımcı et al.
essential to thoroughly examine the endolarynx, including a careful grading of the
CSS [40, 55].
The standard treatment for a newborn with subglottic stenosis is to perform a
tracheostomy and evaluate the infant’s airway every 3 months to determine if
reconstructive surgery is necessary. Under general anesthesia, a cricoid split can be
performed anteriorly or posteriorly to widen the cricoid lumen. Laryngotracheal
reconstruction is the gold standard treatment for subglottic stenosis that has developed over time. In laryngotracheal reconstruction, the larynx is exposed from the
hyoid above to the tracheostomy below by a second incision made above the tracheostomy at the cricoid level. The larynx is incised precisely down the middle,
and the lumen is entered above the superior thyroid notch. A graft of costal cartilage widens the cricoid ring. The graft is kept from collapsing with the use of a
silastic stent. It is crucial to precisely oppose the anterior commissure during incision closure [56].
4.4.6 Subglottic Hemangioma
Premature infants often develop subglottic infantile hemangiomas, a benign vascular abnormality. The occurrence of cutaneous hemangiomas in more than half of
patients with a subglottic hemangioma suggests the presence of a synchronous subglottic lesion [40]. The likelihood of developing a subglottic hemangioma is higher
in patients whose hemangioma is located in the beard distribution (i.e., the chin,
jawline, and preauricular areas) [57]. Infantile hemangiomas occur in females at a
rate of 2:1 compared to males [2]. Pathologically, these tumors are characterized by
uniform, signicantly positive staining for erythrocyte-type glucose transporter protein isoform 1 (GLUT-1) and endothelial proliferation. Biphasic stridor with retractions are symptoms, and they are most noticeable when the youngster is angry or
eating. There may also be a barking cough, like that of croup. Apnea, cyanosis, and
“dying spells” can happen with severe airway obstruction.
Transnasal exible laryngoscopy with the patient awake is preferable for the
initial assessment. It may make it possible to see the damaged subglottis, and more
importantly, it should eliminate other potential causes of neonatal stridor, such as
laryngomalacia and paralysis of the vocal folds. A laryngoscopy and bronchoscopy
performed in the operating room under general anesthesia are necessary for a child
with increasing stridor and a standard glottic and supraglottic evaluation. A radiographic examination of the neck and an MRI scan (T2-weighted gadolinium contrast) can help diagnose the narrowing of the subglottis [2, 40].
Most people need some treatment, and doctors often use multiple approaches.
Subglottic hemangiomas are traditionally treated with systemic steroids, which
often cause at least partial hemangioma regression in most children [2]. Subglottic
hemangiomas can cause airway obstruction, and typical surgical treatment
involves performing a tracheotomy and hoping for spontaneous involution.
Reports of success using a CO2 laser to decannulate a tracheostomy have
been mixed.

4 Congenital Anomalies oftheUpper Respiratory Tract
63
4.4.7 Laryngeal Cysts
Rare causes of respiratory obstruction in children include saccular cysts and laryngoceles [2]. It is located superiorly between the vestibular fold and the inner side of
the thyroid cartilage, and the laryngeal saccule is a tiny diverticulum containing
many mucous glands at the anterior end of the laryngeal ventricle [58]. Mucus-lled
congenital saccular cysts extend laterally into the false vocal and aryepiglottic fold.
In the initial few days of birth, babies with these cysts often have trouble breathing,
their cries are muted, and they have trouble swallowing [59]. Internal, external, or
combined dilatation and herniation of the saccule characterize a laryngocel.
Sometimes there are air-uid levels within laryngoceles, giving the appearance of
spherical lumps in the neck or supraglottic region [2]. Saccular cysts generate persistent symptoms due to mucoid uid within the cyst, whereas laryngoceles cause
occasional upper airway blockage and hoarseness due to episodic lling with air.
The results of needle aspiration, marsupialization, or endoscopic excision for
managing saccular cysts have historically been unsatisfactory, often necessitating
repeated surgeries and tracheotomy installation. The author prefers an anterior
cervical approach, with the superior border of the thyroid alar cartilage being
found and the thyrohyoid membrane being incised along its superior border. This
method allows the cyst to be reached and carefully dissected free before removal.
Thyroid cartilage may be removed to facilitate cyst removal and reattachment
[40, 59].
4.4.8 Laryngeal Cleft
Clefts of the larynx, esophagus, or windpipe are extremely unusual congenital disabilities. Failure of the laryngotracheal groove to fuse during development causes
laryngeal clefts [2]. Associated defects affecting the patient’s airway or other organ
systems are common. Tracheomalacia (present in >80% of patients) and tracheoesophageal stula (TEF) formation (present in 20% of patients) are two of the most
common airway abnormalities seen in these patients. GER and anogenital abnormalities have been linked to a lack of airway development. Opitz-Frias syndrome,
which includes hypertelorism, anogenital abnormalities, and posterior laryngeal
clefting, is the most prevalent syndrome involving this congenital disability [4].
Benjamin and Inglis [2, 60] suggested a helpful anatomic classication and divide
posterior laryngeal clefts into the following four subtypes [2, 60]:
Type 1: supraglottic inter arytenoid cleft present to, but not below, the level of the
true vocal folds. This cleft could also be considered a deep inter arytenoid notch.
Type 2: Partial cricoid cleft that extends into, but not through, the posterior cricoid
cartilage.
Type 3: Total cricoid cleft with or without extension of the cleft into the cervical
trachea.
Type 4: Cleft extending to the thoracic trachea [2, 60].

64
E. K. Yardımcı et al.
Coughing (particularly during eating), difculty feeding, and respiratory discomfort (depending on the depth of the split) are some of the earliest signs of a
laryngeal cleft. Cyanosis (especially in those with other underlying health problems
or abnormalities), aspiration, recurrent lung infections, stridor, wheezing, failure to
thrive, and cyanosis are some of the other symptoms that may be present. Clefts of
types III and IV are linked to more severe respiratory symptoms, including frequent
episodes of pneumonia and increased mucus production. In most cases, types III
and IV symptoms appear within the rst week of life [2, 60].
Rigid bronchoscopy and esophagoscopy are the gold standard for diagnosis. In contrast, other methods such as exible nasopharyngoscopy, beroptic endoscopic evaluation of swallowing, video uoroscopy-barium swallow, and exible bronchoscopy are
helpful. The cleft is frequently missed or misinterpreted during initial evaluation with
exible or rigid bronchoscopy due to redundant mucosa in the posterior glottis [40].
Several surgical methods have been explored in recent decades for treating laryngeal cleft. In 1967, Evans [61] stated that Jahrsdoerfer and colleagues described the
anterior method, which entails exposing the cleft via thyrotomy, cricoidotomy, and
trachea-ssure to the rst and second rings to provide direct access to the posterior cleft
and permit microsurgical closure. Due to the potential for laryngeal instability and
long-term laryngeal development problems, some publications have recommended
avoiding the anterior approach [61, 62]. Avoiding the laryngossure has also been
described for the lateral pharyngotomy approach [61]. According to other researchers
[63], the risk of laryngeal instability with the anterior approach can be reduced with
careful closure under magnication, correct stenting with a nasotracheal tube, and
appropriate post-operative care in the intensive care unit. A lateral pharyngotomy with
a right thoracotomy approach or an anterior approach with a median sternotomy has
been employed for a more extensive cleft involving the thoracic trachea [64].
4.5 Conclusion
Assessing a newborn with respiratory distress requires expert knowledge of upper
airway embryological development and congenital abnormalities. Most infants
experiencing respiratory distress (58%) were in the 1–6 months age range.
Laryngomalacia, subglottic stenosis, laryngeal web, and vocal fold paralysis were
not strongly associated with age, sex, family history, or other congenital disabilities.
Diagnosis is typically made through endoscopy. The severity of an illness determines how it is treated.
References
1. Daniel SJ. The upper airway: congenital malformations. Pediatr Respir Rev. 2006;7(Suppl
1):S260–3.
2. Truong MT, Messner AH.Evaluation and management of the pediatric airway. In: Cummings
pediatric otolaryngology. Elsevier; 2015. p.309–22.
3. Flake CG, Ferguson CF.Congenital choanal atresia in infants and children. Ann Otol Rhinol
Laryngol. 1964;73:458–73. https://doi.org/10.1177/000348946407300216.

4 Congenital Anomalies oftheUpper Respiratory Tract
4. Ramsden JD, Campisi P, Forte V.Choanal atresia and choanal stenosis. Otolaryngol Clin N
Am. 2009;42(2):339–52.
5. Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classication and
clinical management applications. Laryngoscope. 1996;106(1 Pt 1):97–101. https://doi.
org/10.1097/00005537- 199601000- 00019.
6. Kwong KM.Current updates on choanal atresia. Front Pediatr. 2015;3:52.
7. Andaloro C, La Mantia I.Choanal atresia. StatPearls. Last update 27 Sept 2020.
8. Brown OE, Myer CM, Manning SC. Congenital nasal pyriform aperture stenosis.
Laryngoscope. 1989;99:86–91.
9. Ey EH, Han BK, Towbin RB, Jaun WK.Bony inlet stenosis is a cause of nasal airway obstruction. Radiology. 1988;168:477–9.
10. Belden CJ, Mancuso AA, Schmalfuss IM.CT features of congenital nasal piriform aperture
stenosis: initial experience. Radiology. 1999;213:495–501.
11. Devambez M, Delattre A, Fayoux P.Congenital nasal pyriform aperture stenosis: diagnosis
and management. Cleft Palate Craniofac J. 2009;46:262–7.
12. Mulliken JB.Bilateral complete cleft lip and nasal deformity: an anthropometric analysis of
staged to synchronous repair. Plast Reconstr Surg. 1995;96:9.
13. Rutter MJ.Evaluation and management of upper airway disorders in children. Semin Pediatr
Surg. 2006;15:116–23.
14. Rand PK, Ball WS Jr, Kulwin DR. Congenital nasolacrimal mucoceles: CT evaluation.
Radiology. 1989;173:691–4. https://doi.org/10.1148/radiology.173.3.2813773.
15. Shashy RG, Durairaj VD, Holmes JM, Hohberger GG, Thompson DM, Kasperbauer
JL.Congenital dacryocystocele associated with intranasal cysts: diagnosis and management.
Laryngoscope. 2003;113:37–40. https://doi.org/10.1097/00005537- 200301000- 00007.
16. Yin T, van der Meer G.Neonatal airway obstruction in bilateral congenital dacryocystocoele:
case report and review of the literature. Int J Pediatr Otorhinolaryngol. 2017;92:161–4. https://
doi.org/10.1016/j.ijporl.2016.11.027.
17. Kallen K. Maternal smoking, body mass index, and neural tube defects. Am J Epidemiol.
1998;147:1103–11.
18. Rahbar R, Resto VA, Robson CD, Perez-Atayde AR, Goumnerova LC, McGill TJ, etal. Nasal
glioma and encephalocele: diagnosis, and management. Laryngoscope. 2003;113:2069–77.
19. Phillips CD.Neuroradiologic imaging in craniofacial surgery. In: Lin KY, Ogle RC, Jane JA,
editors. Craniofacial surgery: science and surgical technique. Philadelphia: Saunders; 2002.
p.153–60.
20. Marshall AH, Jones NS, Robertson IJA.Endoscopic management of basal encephaloceles. J
Laryngol Otol. 2001;115:545–7.
21. Hughes GB, Sharpino G, Hunt W, etal. Management of the congenital midline nasal mass—a
review. Head Neck Surg. 1980;2:222–33.
22. Messner AH, Richardson MA, editors. Cummings otolaryngology-head and neck surgery.
23. Littlewood AH.Congenital nasal dermoid cysts and stulas. Plast Reconstr Surg Transplant
Bull. 1961;27:471–88.
24. Ni K, Li X, Zhao L, Jiali W, Liu X, Shi H.Diagnosis and treatment of congenital nasal dermoid
and sinus cysts in 11 infants. Medicine (Baltimore). 2020;99(21):e19435. Published online
2020 May 22. https://doi.org/10.1097/MD.0000000000019435.
25. Pierre Robin syndrome. MedlinePlus. 29 Oct 2013. http://www.nlm.nih.gov/medlineplus/
ency/article/001607.htm.
26. Isolated Pierre Robin sequence. Genetics Home Reference (GHR). Aug 2013. https://ghr.nlm.
nih.gov/condition/isolated- pierre- robin- sequence.
27. Treacher Collins syndrome. Genetics Home Reference. Jun 2012. http://ghr.nlm.nih.gov/con-
dition/treacher- collins- syndrome. Accessed 27 Oct 2014.
28. Vazquez M-P.Treacher Collins syndrome. Orphanet. Jan 2014. http://www.orpha.net/consor/
cgi- bin/OC_Exp.php?lng=en&Expert=861. Accessed 27 Oct 2014.
29. Crouzon syndrome. Genetics Home Reference. Feb 2008. http://ghr.nlm.nih.gov/condition/
crouzon- syndrome. Accessed 7 Nov 2015.
65

66
30. Arnaud E, Collett C, Di Rocco F.Crouzon disease. Orphanet. Nov 2013. http://www.orpha.
net/consor/cgi- bin/OC_Exp.php?lng=en&Expert=207.
31. O’Neill MJF.Crouzon syndrome. OMIM. 18 Mar 2016. http://www.omim.org/entry/123500.
32. Shott SR. Down syndrome: common otolaryngologic manifestations. Am J Med Genet.
2006;142C:131. https://doi.org/10.1002/ajmg.c.30095.
33. Gazi SR, Manu PS, Vijayalakshmi S. Apert’s syndrome—a rare craniofacial anomaly.
Southeast Asian J Case Rep Rev. 2014;3:645–67.
34. Satyanand T, Sachin K, Mohit S.Etiology, symptoms and treatment of Apert syndrome, a
congenital disorder: an overview. Int J Pharm Bio Sci. 2010;1:1–7.
35. Garcia E, Osterbauer B, Parham D, Koempel J.The incidence of microscopic thyroglossal
duct tissue superior to the hyoid bone. Laryngoscope. 2019;129(5):1215–7.
36. Ma J, Ming C, Lou F, Wang ML, Lin K, Zeng WJ, Li ZC, Liu XF, Zhang TS.Misdiagnosic
analysis and treatment of pyriform sinus stula in children. Zhonghua Er Bi Yan Hou Tou Jing
Wai Ke Za Zhi. 2018;53(5):381–4.
37. Ross J, Manteghi A, Rethy K, Ding J, Chennupati SK.Thyroglossal duct cyst surgery: a tenyear single institution experience. Int J Pediatr Otorhinolaryngol. 2017;101:132–6.
38. Povey HG, Selvachandran H, Peters RT, Jones MO.Management of suspected thyroglossal
duct cysts. J Pediatr Surg. 2018;53(2):281–2.
39. Pucher B, Jonczyk-Potoczna K, Kaluzna-Mlynarczyk A, Kurzawa P, Szydlowski J.The central
neck dissection or the modied Sistrunk procedure in the treatment of the thyroglossal duct
cysts in children: our experience. Biomed Res Int. 2018;2018:8016957.
40. Rutter MJ.Congenital laryngeal anomalies. Braz J Otorhinolaryngol. 2014;80(6):533–9.
41. Richter GT, Wooten CT, Rutter MJ, Thompson DM.Impact of supraglottoplasty on aspiration
in severe laryngomalacia. Ann Otol Rhinol Laryngol. 2009;118:259–66.
42. Loke D, Ghosh S, Panarese A, etal. Endoscopic division of the aryepiglottic folds in severe
laryngomalacia. Int J Pediatr Otorhinolaryngol. 2001;60:59–63.
43. Senders CW, Navarete EG. Laser supraglottoyplasty for laryngomalacia; are specic anatomical defects more inuential than associated anomalies on the outcome? Int J Pediatr
Otorhinolaryngol. 2001;57:235–44.
44. Gandhi S, Oswal V, Thekedar P, etal. Role of transoral CO2 laser surgery for severe pediatric
laryngomalacia. Eur Arch Otorrinolaringol. 2011;268:1479–83.
45. Miyamoto RC, Parikh SR, Gellad W, Licameli GR.Bilateral congenital vocal cord paralysis: a
16-year institutional review. Otolaryngol Head Neck Surg. 2005;133:241–5.
46. Choi SS, Tran LP, Zalzal GH.Airway abnormalities in patients with Arnold-Chiari malformation. Otolaryngol Head Neck Surg. 1999;121:720–4.
47. Ada M, Isildak H, Saritzali G. Congenital vocal cord paralysis. J Craniofac Surg.
2010;21(1):273.
48. Hartnick CJ, Brigger MT, Willging JP, Cotton RT, Myer CM 3rd. Surgery for pediatric vocal
cord paralysis: a retrospective review. Ann Otol Rhinol Laryngol. 2003;112:1–6.
49. Trey LA, Lambercy K, Monnier P, Sandu K.Management of severe congenital webs-12 year
review. Int J Pediatr Otorhinolaryngol. 2016;86:82–6.
50. Cohen SR.Congenital glottic webs in children. A retrospective review of 51 patients. Ann Otol
Rhinol Laryngol Suppl. 1985;121:2–16.
51. Avelino MAG, Pazinatto DB, Rodrigues SO, Maunsell R.Congenital laryngeal webs: from
diagnosis to surgical outcomes. Braz J Otrhinolaryngol. 2022;88:497.
52. Hartnick CJ, Cotton RT. Congenital laryngeal anomalies. Laryngeal atresia, stenosis, webs,
and clefts. Otolaryngol Clin N Am. 2000;33(6):1293–308.
53. Tesmera P, Wróblewska-Seniuka K, Mazelaa J, Szydłowskib J.Congenital laryngeal stenosis
and concomitant birth defects in a term newborn: a case report. Biomed Hub. 2020;5:1.
54. Blanchard M, Leboulanger N, Thierry B, Blancal JP, Glynn F, Denoyelle F, etal. Management
specicities of congenital laryngeal stenosis: external and endoscopic approaches.
Laryngoscope. 2014;124(4):1013–8.
55. Myer CM 3rd, O’Connor DM, Cotton RT.The proposed grading system for subglottic stenosis
is based on endotracheal tube sizes. Ann Otol Rhinol Laryngol. 1994;103:319–23.
E. K. Yardımcı et al.

4 Congenital Anomalies oftheUpper Respiratory Tract
56. Morrissey MS, Bailey CM.Diagnosis and management of subglottic stenosis after neonatal
ventilation. Arch Dis Child. 1990;65(10):1103–4.
57. Orlow SJ, Isakoff MS, Blei F.Increased risk of symptomatic hemangiomas of the airway in
association with cutaneous hemangiomas in a beard distribution. J Pediatr. 1997;131:643–6.
58. Janfaza P, Montgomery WW, Randolph GW.Anterior regions of the neck. In: Janfaza P, Nadol
JB, Galla R, Fabian RL, Montgomery WW, editors. Surgical anatomy of the head and neck.
Philadelphia, PA: Lippincott Williams & Wilkins; 2001. p.629–74.
59. Cotton RT, Prescott CAJ.Congenital anomalies of the larynx. In: Cotton RT, Myer III CM,
editors. Practical pediatric otolaryngology. Philadelphia, PA: Lippincott-Raven Publishers;
1999. p.497–513.
60. Benjamin B, Inglis A.Minor congenital laryngeal clefts: diagnosis and classication. Ann Otol
Rhinol Laryngol. 1989;98:417–20.
61. Evans JG. Management of the cleft larynx and tracheoesophageal clefts. Ann Otol Rhinol
Laryngol. 1985;94:627–30.
62. Donahoe PK, Gee PE.Complete laryngotracheoesophageal cleft: management and repair. J
Pediatr Surg. 1984;19:143–8.
63. Froehlich P, Truy E, Stamm D, Morgan A, Floret D, Chappuis JP.Cleft larynx: management
and one-stage surgical repair by anterior translaryngotracheal approach in two children. Int J
Pediatr Otorhinolaryngol. 1993;27:73–8.
64. Cotton RT, Schreiber JT.Management of laryngotracheoesophageal cleft. Ann Otol Rhinol
Laryngol. 1981;90:401–5.
67

Immunological Defense Mechanisms
oftheRespiratory System
NeslihanEdeerKaraca, AyseAygun, andTsvetelinaVelikova
The respiratory tract continuously faces inhaled air containing microbial organisms,
airborne pollutants, particles, noxious gases, and allergens. The pulmonary immune
system must also cope with potentially harmful threats and react rapidly to protect
the host [1–3]. Different physical, secretory, and cellular factors are involved in the
immune response in the lung parenchyma and airways, constituting the two functional areas of the respiratory system.
The airway epithelium and submucosa serve as the respiratory primary defense
system as both a mechanical and immunological barrier [4]. Pulmonary host defense
has evolved innate and specic immune mechanisms. The airway epithelium mainly
comprises ciliated columnar cells and secretory (goblet) cells that provoke mucociliary elimination of inhaled antigens. Apart from providing a physical barrier, the epithelium is immunologically active by secreting diverse antimicrobial peptides,
releasing chemokines, cytokines, and growth factors, modulating adaptive immunity,
and remodeling with tissue repair. In the mucosa, there are macrophages, dendritic
cells (DCs), plasma cells, innate lymphoid cells (ILC), natural killer (NK) cells, and
T lymphocytes [1–3]. Epithelial T lymphocytes in the mucosa are mainly CD8+ T
cells; in the lamina propria, CD4+ T cells are abundant [1, 3, 5]. There are also areas
of lymphoid tissue (LT) in the airway mucosa, namely, nasal mucosa-associated LT
(NALT) and bronchus-associated LT (BALT) [6]. This tissue is not encapsulated and
is in direct contact with epithelial mucosa. In the lung parenchyma, there are alveolar
macrophages and small proportions of T and B lymphocytes and dendritic cells.
Innate and adaptive immunity have signicant protective and therapeutic roles
against infections and inammation in the respiratory tract (Fig.5.1).
5
N. E. Karaca (*) · A. Aygun
Department of Pediatric Immunology, Ege University Faculty of Medicine, Izmir, Türkiye
T. Velikova
Soa University, Faculty of Medicine, “St. Kliment Ohridski”, Soa, Bulgaria
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2024
H. Yüksel et al. (eds.), Pediatric Airway Diseases, Comprehensive ENT,
https://doi.org/10.1007/978-3-031-74853-0_5
69

70
N. E. Karaca et al.
Fig. 5.1 Immune response in the respiratory tract. Mucosa in the upper and lower airways is constantly exposed to environmental and infectious agents, such
as LPS, agellin, pilin, and other bacterial antigens, as well as various allergens (dust, etc.). Epithelial cells are central in defending respiratory mucosal mem-
brane by both secreting type I and II interferons, lactoferrin, defensins, nitric oxide, etc. into the airway lumen, and communicating with the immune cells in
the mucosa by releasing chemokines (such as MP-1, IL-8, MCP-1, and RANTES) and cytokines (IL-6, IL-1b, TNF-α, etc.) to activate immune cells into the
mucosa. Epithelial cells along with macrophages, dendritic cells, mast cells, and neutrophils are the rst line of defense, involved in direct pathogen clearance
(via IFN-γ production, phagocytosis, etc.) and via direct attraction, recruitment, and activation of effector cells (innate lymphoid cells, tissue-resident T cells,
NK, and NKT cells). This two-tiered immune response eventually leads to pathogen clearance and tissue repair. Furthermore, because of the proinammatory
signals, naïve T cells along with FoxP3+ T regulatory cells predominantly differentiate into Th17 cells, which are engaged in the clearance of pathogens. (Parts
of the gure were drawn by using pictures from Servier Medical Art. Servier Medical Art by Servier, licensed under a Creative Commons Attribution 3.0
Unported License. https://creativecommons.org/licenses/by/3.0/)

5 Immunological Defense Mechanisms oftheRespiratory System
71
5.1 Innate Immunity
The initial phase of lung defense involves the innate immune system, including
defensive structures throughout the airway lumen and lung parenchyma, antimicrobial agents, alveolar macrophages, DCs, neutrophils, NK cells, and ILCs [1–3].
1. The airway epithelium is at the interface of the human body between the external
inhaled environment and the internal tissues. It forms a complex physicochemical and mechanical barrier supplemented by mucociliary clearance to provide
the rst line of defense against inhaled pathogens. The airway mucus covering
the respiratory tract’s luminal surface entraps inhaled pathogens, including
infectious agents, and eliminates them by the coordinated beating of the cilia.
Epithelial cells maintain epithelial integrity by attaching to their neighbors by
tight junctions, adherens junctions, gap junctions, and desmosomes [7]. These
structures form an impermeable and effective mechanical barrier to most pathogens and permit the maintenance of an ionic gradient for the directional exchange
of many nutrients, water, and gases [7, 8]. The upper airway epithelial cells efciently prevent microparticles >10μm and a large percentage of particles >5μm
from reaching the lower airways via direct prevention of their passage through
the nasopharyngeal mucosa [8, 9]. The particles entrapped are either exhaled
with cough or ingested with mucus into the gastrointestinal tract. If pathogens
crack structural defenses, the innate immune system mediators of the upper
respiratory tract, including salivary lysozyme, peroxidase, and lactoferrin, function with antimicrobial activity. Lower respiratory system airways are also lined
with epithelial cells and beating cilia. Approximately 90% of particles >2–3μm
trapped within the mucus are transported through the mucociliary escalator from
the bronchioles to the trachea and exhaled with the help of a cough [7].
2. The airway mucus is a viscoelastic gel secreted continuously by intraepithelial
goblet cells and by mucous cells of submucosal glands toward the surface of the
epithelium. The airway lining uid is composed of different proteins, such as
mucins and antimicrobial substances (lysozyme, lactoferrin, and defensins),
cytokines, antiproteases, and antioxidant proteins [10–12]. The airway damage
can be induced from microbes as well as from several factors that are produced
during inammatory reactions and phagocytosis. Lysozyme destroys the walls of
the microbes and fungus. It also has the protective role of inhibiting the destructive effects of oxidative factors produced during inammation [13].
Defensins are cationic peptides secreted by neutrophils (α-defensin) and epi-
thelial cells (β-defensins) [10]. These peptides exhibit antimicrobial activity
against various types of bacteria, mycobacteria, fungi, and some enveloped
viruses by promoting chemokine production and subsequent migration of host
inammatory cells, inducing phagocytosis, complement activation, and CD4+
T-cell proliferation [10, 13]. Lactoferin can bind with iron and prevent the utilization of elemental iron by pathogenic bacteria [14, 15].
Collectins, or collagen-like lectins, are another family of small proteins with
important anti-pathogen properties [16]. Their role is to inhibit the invasion of

72
N. E. Karaca et al.
epithelial cells by several respiratory viruses and facilitate the phagocytosis and
subsequent clearance of target microorganisms. These molecules exhibit a wide
range of specicities. They interact with pathogens through their lectin domains
and regulate the functions of T lymphocytes, macrophages, dendritic cells, and
neutrophils. Important collectins are the mannose-binding lectin (MBL) and surfactant proteins A and D [16–18]. Collectins cannot destroy microbes directly;
they are responsible for enhancing macrophage phagocytosis.
3. The respiratory tract microbiome, a collection of microorganisms (bacteria,
viruses, fungi, and archaea) residing in the respiratory mucosa, maintains a symbiotic relationship with the host. It acts as a barrier against the invasion of pathogens and plays an essential role in maintaining the balance between immune
tolerance and inammation [19].
4. Mast cells and plasma cells, which secrete IgA, are among the primary cells
present in the lamina propria. The secretory IgA barrier facilitates the benecial
local microbiome’s permanence and helps to eliminate noxious agents, such as
pathogens and pollutant particles, by inhibiting their adherence to the epithelium [15].
5. Complement, a complex protein system, is a critical component of the innate
immune response. The complement system plays a crucial role in the defense
against invading pathogens through bacterial lysis, stimulation of phagocytosis,
the recruitment of immune cells to infected tissue, and the promotion of the
inammatory response. Although complement is well characterized in the
serum, complement activity is also present in the lung [20]. Alveolar macrophages and pulmonary alveolar type II epithelial cells synthesize and secrete
complement proteins C2, C3, C4, C5, and Factor B, whereas bronchiolar epithelial cells can generate C3 [21]. Once complement is activated by the hydrocarbons on the outer membrane of the microorganisms or the collectins, the cascade
leads to cell death by the nal formation of the lytic membrane attack complex
(C5–C9), which forms pores on the target cell membrane.
6. If the microorganism overcomes the mucociliary escalator and defensive proteins, it reaches its target, the respiratory epithelial cells. As mentioned before,
these cells act as an effective mechanical barrier to the microorganism entry and
dissemination into the submucosa. Respiratory epithelial cells produce antimicrobial and immunomodulatory molecules such as interferons, lactoferrin, and
defensins. These cells express receptors within the basolateral epithelial membrane, serving as an entry site for several viruses. Under steady-state conditions,
the epithelial cells of both the bronchi and the alveoli have essential roles in
maintaining tolerance by producing the anti-inammatory cytokines interleukin
(IL)-10 and TGF-β [19]. In case of exposure to exogenous microorganisms or
tissue damage of any sort, the airway epithelium senses antigens via pattern
recognition receptors (PRR) such as Toll-like receptors (TLR), RIG-I-like receptors (RLR), Nod-like receptors (NLR), and C-type lectin receptors [22–24].
When PRRs recognize the molecular structures common to pathogens, called
pathogen-associated molecular patterns (PAMPs), they activate intracellular
down-stream signaling pathways that ultimately result in the translocation of
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