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Respiratory System
Embryo/disorders
125
Nursing
Graduate
10/30/2011

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Term
Questions
Definition
Answers
Term
When does the respiratory system begin to develop?
Definition
4 weeks gestation
Term
What structures are part of the conducting zone?
Definition
nasal cavity, pharynx, larynx, trachea, bronchi, bronchioles
Term
What structures are part of the respiratory zone?
Definition
respiratory bronchioles, alveolar ducts, alveolar sacs and the alveoli
Term
What structures are cephalic to the larynx?
Definition
nasal and oral cavities, pharynx
Term
The formation of the lung bud is caused by what process?
Definition
lateral folding of two sphlanchnic mesoderm and endoderm that meed at midline to separate the larynx and trachea from the esophagus
Term
The larynx is first seen as what structure?
Definition
ventral outgrowth of endoderm from the foregut --> lung bud/respiratory diverticulum
Term
The larynx arises from what early embryology tissues?
Definition
1. epithelium: endoderm
2. muscles and cartilage: neural crest cells
Term
The trachea develops from what tissues?
Definition
1. epithelium: endoderm
2. muscles/cartilage comes from splanchnic mesoderm
Term
At the end of lung development in early childhood, how many divisions have the bronchi undergone?
Definition
24 generations of branching
Term
List, in order of earliest to latest in development, the branching of the trachea to form bronchioles.
Definition
Trachea --> L/R bronchi --> R bronchus gives off 3 diverticula and L bronchus gives off two diverticula ---> lobular bronchi --> smaller bronchi ---> bronchioles
Term
At birth in a term baby, how many generations of branching has occurred?
Definition
more than 17, but less than 24
Term
Name the 4 stages of development for the lungs
Definition
Pseudoglangular, canclicular, terminal sac, and aveolar period
Term
What happens during the pseudoglandular period?
Definition
-bronchi are dividing into bronchioles. Typically from week 5th-28th week
-trachea and foregut separate
Term
What is the canalicular period
Definition
-formation of respiratory bronchioles (which will give rise to alveolar ducts)
-Epithelium is cuboidal
-capillaries proliferate but do not reach the respiratory Epithelium
-starting at 5th month - type II alvelolar epithelial cells develop
Term
Describe the events of the terminal sac phase of lung development
Definition
-starts at wk 26 - birth
-capillaries proliferate and begin to reach respiratory epithelium
-epithelium becomes squamous epithelium made of type I alveolar epithelial cells
-starting at 5th month - type II alvelolar epithelial cells develop
Term
What cells produce surfactant?
Definition
type II alvelolar epithelial cells?
Term
At what stage in lung development does the fetus become viable? Why?
Definition
during the terminal sac phase - weeks 25/26 and on because type I alveolar cell epithelial cells are capable of gaseous exchange
Term
What occurs during the alveolar period and when does it happen during development?
Definition
-growth of lungs due to mature alveoli. Increase in surface area where alveoli and capillaries meet
-starts at 8th month of development and continues until age 10
Term
What is the septum transversum and how does it develop?
Definition
-separates the throacic cavity and abdominal cavity
-develops from body wall after lateral body folding is completed
Term
What are the pericardioperitoneal canals?
Definition
communication between the abdominal and thoracic cavity that remains
Term
Explain the embryologic developments that occur to result in on peridcardial cavity and two pleural cavities
Definition
-fibrous pericardium forms from the pleuopericardial folds and extends caudally until it inttersects with the septum transversum
-as the pleuropericardial folds attach to the primitive mediastinum it separates the heart from the lungs creating a pericardial cavity and two pleural cavities
Term
The diaphargm develops from which four structures?
Definition
1. lateral body wall mesoderm
2. septum transversum
3. dorsal mesentery of the esophagus
4. pleuroperitoneal folds
Term
Explain the development of the diaphragm
Definition
The diaphrgam develops initially in a more cephalic region and decends caudally into the thoracic position. Phrenic nerves travel with the diaphragm and end up in the fibrous pericardium
Term
Where does the phrenic nerve found?
Definition
embedded in the fibrous pericardium as it travels with the diaphragm during development
Term
What is the diaphragm innerveated by?
Definition
3-5th cervical spinal cord segments via phrenic nerve
Term
What is the earliest lung malformation?
Definition
Agenesis fom fialure of two bronchial buds to form from the larynx (lung bud)
Term
What condition results from an errorrs of development during the pseudoglandular period?
Definition
-TEF or esophageal atresia
-Congenital diaphragmatic hernia & lung hypoplasia
-abnormal bronchial divison disorders (bronchogenic cysts & pulmonary sequestration)
Term
What is the most common type of TEF?
Definition
H type.
Term
What is congenital diaphragmatic hernia?
Definition
herniation of abdominal contents into the chest thorax preventing lung development
Term
Why is CDH most commonly left-sided?
Definition
nothing preventing herniation - liver on R side
Term
What are the assessment findings associated with CDH?
Definition
scaphoid abdomen and respiratory distress
-usually dx prenatally
Term
Why do we give corticosteriods to preterm babies?
Definition
1. cuboidal --> squamous conversion
2. terminal sac development
3. increase in # of bronchial divisions
4. vasculature development (more capillaries - alveoli connections)
Term
Why are steroid induced growth factors necessary?
Definition
to facilitate bronchial divisions, vasculature, epithelium, CT development
Term
What congenital condition results from an error in development during the alveolar period?
Definition
Pulmonary hypoplasia (NOT agenesis)
Term
What is pulmonary hypoplasia?
Definition
-reduced number of bronchioles, terminal bronchioles, alveoli, and arterioles
-often assoc. with Tris 18, 21
Term
What causes pulmonary hypoplasia?
Definition
1. space occupying lesions (CDH, pleural effusion, small thoracic cage
2. Oligohydramnios - Potter syndrome
3. decreased vascular supply (CHD, TOF, tricuspid atresia, subpulmonic stenosis)
Term
At birth, infants have how many alveoli?
Definition
50 million (~5% of total)
Term
By what age does alveolar development stop?
Definition
8 years old. 300million alveloi (~95%)
Term
What three disorders result from abnormal bronchial divisions?
Definition
1. bronchogenic cyst
2. pulmonary paranchymal cyst
3. pulmonary sequestration
Term
What are bronchogenic cysts and what is the treatment prescribed?
Definition
-cartilaginous, fluid filled sacs that result from anomalous cell proliferation and differentiation during budding/division of the bronchial tree
-can occur at any point after divisons start
-early onset (<12 weeks) will result in mediastinal cysts
- late onset will result in peripheral cysts
Tx: surgical resection only if experiencing respiratory compromise
Term
What is pulmonary paranchymal cyst?
Definition
-thin walled, elastic tissue, single, multilocular peripheral cysts that are ~75% air filled
-occurs during canalicular period as terminal bronchioles are developing
Tx: surgical resection
Term
What is pulmonary sequestration?
Definition
-abonromal, accessory bud that retains arterial connections with GI vasculature ==> non fuctional, ectopic lung tissue with no blood supply ==> no gas exchange occurs
Term
What are the different types of pulmonary sequestration?
Definition
1. extralobar (25%)
left sided, subpulmonic and surrounded own/separate pleura.
-may be solid and impair fetal circulation
2. Intralobar (75%)
left-sided, lower lobes w/o separate pleura (within lung lobes)
-often asymptomatic
Term
How is pulmonary sequestration dx and what is the treatment?
Definition
-MRI/MRA and angiography for Dx
-surgical resection - lobectomy or resection en bloc (extralobar sequestration)
Term
What is Cystic adnomatoid malformation (CAM or C-CAM)
Definition
Characterized by:
-abnormal mesenchymal proliferation
-failed maturation of the bronchiolar structures
-adenomatous (CT) overgrowth --> cysts
-cysts communicate directly with tracheobronchial tree and each other
Term
What is congenital lobar emphysema (CLE)?
Definition
overdistention and airtrapping of a lung lobe due to a ball-valve like mechanism
-more common in males, 20% are bilateral, fairly uncommon
Term
What are the common causes of CLE?
Definition
-deficiency of cartilate
-enlarged vascular structure
-overlying mass or intrathroacic mass from lymphadenopathy or bronchogenic cyst
Term
What is the role of surfactant in preventing atalectasis?
Definition
-the water molecules of alveolar fluid is attracted to the water molecules in the air favoring lung collapse
*surfactant breaks the surface tension between these water molecules by disrupting water molecule bonds
==> increases lung compliance
Term
What is surfactant made of?
Definition
phospholipids and proteins
Term
What stimulates surfactant production?
Definition
stretching of the air spaces with berathing
Term
During what period do fetal breathing movements occur?
Definition
alveolar period. Breathin movements condition respiratory muscles and stimulates alveolar development/growth
Term
What is congenital alveolar proteinosis?
Definition
-Surfactant Protein B deficiency
-airway collapse impending because of decreased lung compliance
-increased risk of infection as well
-can cause chronic lung disease (mild) or require lung transplant
Term
Explain meconium aspiration and the neonate
Definition
- meconium may be passed in utero during fetal distress
-meconium is lipid and protein rich and will be irritating to the airway
-if inhaled during birth - causes chemical pneumonitis
-if aspirated into distal airways: inactivates surfactant
==> obstruction via ball-valve like mechanism
Term
What is persistent pulmonary hypertension of the newborn and why does it occur?
Definition
-at birth and in the first few weeks of life, PVR should decrease to allow for adequate blood flow
-IF PVR fails to decrease: R-->L shunt across PDA and/or PFO
Term
Why does hypoxemia result from PPHN?
Definition
decreased pulmonary perfusion because of high PVR
Term
What are the long-term effects of persistent pulmonary HTN?
Definition
1. hypoxemia
2. decreased cardiac output if no cardic shunt exists
3. cor pulmonale
Term
What are the treatment options for pulmonary hypertension?
Definition
1. correct lesion or underlying disease
2. promote pulmonary artery vasodilation (NO/prostacyclins)
3. increase myocardial contraction
4. diuresis
Term
What is PDA and why does it occur?
Definition
-when closure of DA fails to take place because of high pulmonary vascular resistance
Term
What are the effects of PDA and PFO?
Definition
on-going shunting of aortic blood to the lungs or from L-R atria results in pulmonary over-circulation. Long term: pulmonary edema and a fixed increase in pulmonary vascular resistance
Term
What are the characteristics of lung diesease of prematurity?
Definition
RESPIRATORY DISTRESS SYNDROME
-surfactant deficiency due to immature type II pneumocytes
-decreased number or absence of alveoli
-underdeveloped terminal sacs
-thicker/cuboidal epithelium
-wide area between air-spaces and capillaries
POOR OXYGENATION/VENTILATION
Term
What is bronchopulmonary dysplasia?
Definition
-long term sequelae
-arrested lung development bc large and underdeveloped alveoli are less capable of gas exchnage
-reactive bronchi and bronchiole smooth muscle causes obstruction
-inflammation and pulmonary edema
-if severe: pulmonary HTN, interstitial fibrosis with cystic changes
Term
What is the treatment of Lung disease of prematurity?
Definition
-Surfactant instillation
-mecahnical ventilation and supplemental oxygen
-bronchodilators
-steroids
-diurectics
-long term ventilation via trach bc of chronic respiratory failure
Term
Define: ventilation, gas exchange, oxygenation, transport, and regulation.
Definition
ventilation = flow of air in and out of the lungs
gas exchange = diffusion of O2 and CO2
oxygenation: movement of oxygen into the circulation
transport: movement of o2 and co2 via blood and circulatory systems
regulation: control of breathing patterns
Term
What structures belong to the extra-thoracic part of the conducting zone? The intra-thoracic?
Definition
Extra-thoracic:
-nose/mouth, trachea, larynx, pharynx
Intra-thoracic:
-bronchi & bronchioles
Term
What is airway resistence and how is it determined?
Definition
-determined by diameter of the conducting zone structures.
- R= 1/r^4
Term
Describe the mucous lining and cilia characteristics of of the nose --> terminal bronchioles
Definition
1. globet cells that secrete mucous
2. ciliated epithelium with cilia that constantly beat
Term
Where is the "upper airways" and what structures are inclded?
Definition
-everything above the clavicles/throacic inlet. Extra-throacic parts of conducting zone
-mouth, nose, nasopharynx, soft palate, hypopharynx, pharynx, epiglottis, glottis, larynx, proximal trachea
Term
What are important considerations regarding the upper airways?
Definition
-must remain patent
-must have functioning ciliated epithelium and produce mucous
Term
What is the purpose of the upper airways?
Definition
-move air into the lungs
-humidifying an cleaning debris from air entering the lungs
Term
What are the developmental differences in the upper airways between a child and an adult?
Definition
- more narrow diameter (less tolerance for swelling or occlusion)
- more acute angle of upper airway structures in the infant (facilitates nursing)
-narrowing at the level of the glottis (no cuff needed for ET tubes)
Term
Name the common nasal disorders.
Definition
-Infectious Rhinitis
-allergic rhinitis (acute/chronic)
-nasal polyps (CF)
-sinusitis (may be associated with Kartagener Syndrome)
Term
What is olfactory neuroblastomas?
Definition
-highly malignant tumors composed of neuroblasts encircled by vascularized CT
-located superiorly and laterally in the nose
-originates from neuroendocrine cellsd dispersed in olfactory mucosa
Term
What are the common assessment findings of nasopharyngitis, tonsilitis, and laryngitis?
Definition
-reddedning and slight edema of the mucosa with enlargement of related lymph nodes
-tonsils may be enlarged and covered by exudate
-hoarse voice (laryngitis)
Term
What causes nasopharyngitis, tonsilitis, and laryngitis?
Definition
-usually viral in origin (Rhinovirus, echovirus, adenoviruses, RSV, influenza)
-bacterial infections may be superimposed on viral
Term
What is stridor and what causes it?
Definition
-high pitched whilstling on inspiration due to increased resistance in the extra-thoracic airways
-Extra-thoracic airways are not under the influence of negative intrathroacic pressure during inhalation and so are prone to collapse during inhalation causing stridor
Term
What Is are the common assessment findings of peritonsillar abscess?
Definition
-muffled voice
-drooling
-stridor
-trismus
-will have jaw movement
Term
When do peritonsillar abscesses typically form?
Definition
early in the second decade of life (early teens)
Term
What causes peritonsillar abscess?
Definition
most often bacterial in origin
-staph aureus, micrococcus aureus, beta-hemolytic strep and anaerobic bacteria
Term
Compare/contrast pertonsillar and retropharyngeal abscess
Definition
Similarities:
-caused by same bacteria (staph aureus, micrococcus aureus, beta-hemolytic strep and anaerobic bacteria)
-common symptoms: muffled voice, drooling, stridor, trismus
Differences:
-PA occurs in second decade of life
-RA occurs >6years old
-RA symptoms include fever, dysphagia
-RA has difficulty opening mouth - direct laryngoscopiy difficult
Term
What does retropharyngeal abscess look like on an Xray image?
Definition
prominent soft tissue swelling of the posterior pharynx. Much larger space than normal
Term
What is a primary concern in treating someone with epiglottitis?
Definition
Airway obstruction --> EMERGENCY
Term
What causes epiglottitis?
Definition
haemophilus influenza B (vaccine), bacterial infection, Candida
-between 2-7yrs
Term
What are the s/s of epiglottitis?
Definition
S/S:
-rapid onset (<24 hrs)
-high fever
-no cough
-sore throat
-stridor
-muffled voice
-drooling
-toxic apperance
classic symptom: tripod position. DO NOT MOVE PT head TO ASSESS AIRWAY.
Term
What is laryngeal papillomatosis and what causes this condition?
Definition
-perinatal infection with HPV
-onset: infancy - 4 years
Term
What is the most common congenital anomaly of the airway?
Definition
laryngomalacia and tracheomalasia
Term
What are some common s/s of laryngomalacia & tracheomalasia
Definition
-inspiratory stridor that worsens with crying
-hypercapnea (hypoventilation)
-bronchoscopy:arytenoid cartilages and all supraglottic structures collapse inward (toward glottis)
Term
What is laryngomalacia/tracheomalasia?
Definition
Trachea rings not strong enough to maintain patent airway during inhalation
Term
What is a laryngeal web and what is the recommended treatment?
Definition
-congenital condition that results in a thin membrane of soft tissue Typically at the level of the glottis
Treatment: surgical resection bc will present with respiratory distress at birth
Term
Describe the vocal cord movement during inspiration/expiration
Definition
-cords are open during inspiration/phonation
-cords are closed during exhalation and at rest
Term
Where are the vocal cords located?
Definition
protrude from the lateral walls of the glottis and are stretched and posistied by muscles, ligaments, and cartilage
Term
What happens to the vocal cords to during phonation?
Definition
-the vocal cords move together and are stretched sot aht they vibrate when air moves through
-glottic muscles rotate the thyroid and arytenoid cartilages
Term
Describe vocal cord paralysis
Definition
-may be due to congenital or acquired abnormality of the superior laryngeal nerve and or recurrent laryngeal nerven off of the vagus nerve
-assoc with bain stem injuries, brain tumors, chiari malformations, trauma/birth trauma, increased ICP
-may also result from arytenoid cartilage dislocation and vocal cord hematoma assoc with ETT placement
Term
What condition results from upper airway trauma.
Definition
-compression or direct trauma on tracheal mucosa cuases edema, necrosis, and granuloma formation
-induces subltottic stenosis, laryngotracheomalacia, vocal cord injury
Term
What is a common example of upper airway trauma?
Definition
post-extubation extrathoracic airway obstruction - 35%
Term
What is laryngotracheobronchitis?
Definition
-also known as Croup
-often occurs seasonally in the winter in 3mos to 3years
Term
What are the symptoms of croup?
Definition
-gradual onset - low grade fever
- bark-like cough
-inspiratory stridor
-no sore throat/normal voice/no drooling, non-toxic appearance
Term
What causes croup?
Definition
virus - parainfluenza virus A, adenovirus, RSV
Term
What is the classic sign of croup on an xray?
Definition
"steeple sign" with narrowing of traceal air column at the larynx and distension at the hypopharynx
Term
What is tracheitis and when does it occur?
Definition
-infection/inflammation of the trachea
Term
What are the symptoms of tracheitis?
Definition
-high fever (higher than that assoc with croup)
-stridor/bark-like cough
-no sore throat/normal or hoarse voice
-toxic appearance
-purulent sputum
-stridor that does not improve with alpha/beta agonist (inhaled racemic epinephrine)
-supplemental O2 requirement but no signs of pneumonia, bronchitis, bronchiolitis
Term
What is wheezing?
Definition
high pitched whislting sounds on exhalation due to obstruction in intrathoracic airways
Term
Compare the extra-thoracic airway vs th intra-thoracic airway
Definition
intra-thoracic airway is under negative pressure of intrathoracic cavity during inhalation but are prone to obstruction during exhalation --> wheezing
extrathoracic airway is not under the negative pressure of the intrathroacic cavity and is prone to collapse during inhalation
Term
Describe the difference in cartilage formation in the trachea, bronchi, and bronchioles
Definition
Trachea: cartilage rings
Bronchi: cartilage plates
Bronchioloes: no cartilage and depend on transpulmonary pressures to remain open
Term
Describe the smooth muscle structure in bronchi and bronchioles
Definition
Bronchi:
-smooth muscles in areas wihtout cartilage plates
Bronchioles
-almost exclusively made of smooth muscle
Term
Describe the innervaiton of bronchi and bronchioles
Definition
-sympathetic nerve fibers that act on beta 2 receptors
-parasympathetic nerve fibers of vagus nerve
Term
What is the effect of epinephrine on the bronchi/bronchioles
Definition
acts on beta-2 receptors to cause vasodilation
Term
Vasoconstriction in bronchial smooth muscle is caused by what substances?
Definition
acetylcholine, leukotriences (phosphase lipase A & arachidonic acid metabolism), histamine (anaphylaxis)
Term
What are the different disorders of the intrathroacic conducting zone airways?
Definition
bronchitis, bronchiolitis, asthma, bronchiectasis
Term
What is bronchitis and what is it caused by?
Definition
-inflammation of the bronchi
-Cause: viral or bacterial infection; may be allergic or irritant
-typically non-obstructive and produces exudate
Term
What is bronchiolitis and how is it caused?
Definition
-constriction and obstruction with mucous, causing distal atelectasis
-Cause: mostly viral (RSV)
-OBSTRUCTIVE respiratory disease - impairs ventilation
-cilia is denuded- impairing airway clearance
-if affects respiratory bronchioles and alveoli --> pneumonitis and impairs oxygenation
Term
What are the signs and symptoms of bronchiolitis?
Definition
-cough
-wheezing
-prolonged exhalation
-decreased breath sounds
-coarse breath sounds
-tachypnea, increased WOB
-hypercarbia, maybe hypoxemia if terminal bronchioles are involved
-fever/concurrent conjunctivitis or otitis media
Term
What is asthma?
Definition
allergic, hypersensitive, inflammatory and obstructive lung disease
*bronchial constriction with swelling and mucous production
Term
What immunglobulin mediates asthma reactions?
Definition
IgE mediated inflammation: arachidonic acid, leukotrienes, acetylcholine, histamine, prostaglandin D2, platelet aggregating factor, various cytokines
Term
What are the effects of asthma?
Definition
-air trapping/hyperinflation with adjacent atalectasis
-hypercarbia
-hypoxemia when gas exchange is impaired
Term
What is the long-term effect of chronic asthma?
Definition
airway remodeling
Term
Describe the pathology of asthma
Definition
-IgE mediated inflammation
-increased number and hypertorphy of mucus cells/mucus production
-mucus plugs shed contain whorls of shed epithelium
-bronchial walls infiltrated with eosinophils and macrophages
-increased mast cell activity and histamine release
Term
Describe the process of airway remodeling in asthma
Definition
-Accumulation of mucus: increase in number of globlet cells in the mucosa and hypertrophy of submucosal mucus glands
-chronic inflammation; recruitment of eosinophils, macrophages, and other inflammatory cells
-basement membrane underlying the mucosal epithelium is thickened
-hypertrophy and hyperplasia of smooth muscle cells
ALL LEAD TO FIXED BRONCHIAL CONSTRICTION
Term
What are the pharmacologic treatments for asthma?
Definition
Bronchodilators and anti-inflammatory agents
Term
What are some examples of bronchodilators and how do they work?
Definition
-beta-2 agonists stimulate sympathetic response (albuterol, racemic ephinephrine, IV terbutaline)
-anticholinergics: block vagal/parasym response (ipraroprium chloride - Atrovent)
-magnesium sulfate: direct smooth muscle relaxant
Term
What anti-inflammatory agents are used to treat asthma? How do they work?
Definition
-steroids: block mediatros of inflammation and stabalizes mast cells (methylprednisolone or prenisolone and inhaled fluticasone)
-mast cell stabalizers - prevents histamine release (Cromolyn sodium - intal
-leukotriene inhibitors (Montelukast sodium - singulair)
Term
Explain the role of leukotrienes in asthma reactions
Definition
cell membranes injured --> phospholipids generate phospholipases --> arachidonic acid (AA) synthesis --> activates eicosonoid enzymes: cyclooxygenase and lipoxygenase --> lipoxygenase catalyzes the formation of leukotrienes --> bind to G protein coupled receptors that mediate bronchospasm and increased capillary permeability
Term
What is bronchiectasis and who does it affect?
Definition
-permanent dilation of bronchi and bronchioles caused by destruction of the muscle and elastic tissue resulting from chronic bronchitis/bronchiolitis
-common in kids with CF
Term
What conditions can lead to bronchioectasis?
Definition
Congenital/hereditary
-CF, intralobar sequestraion of the lung, immunodef states, Kartagener syndrome
Postinfectious conditions
-necrotizing pneumonia
Bronchial obstruction:
-tumor, foreign bodies, mucus impaction
Other:
-rheumatoid arthritis, SLE, IBD, chronic lung transplant rejection, chronic GVHD after bone-marrow transplant
Term
Explain the general pathway that connects cystic fibrosis and bronchiectasis
Definition
CTFR defect --> impaired mucous clearance --> chronic bacterial infection --> chronic neutrophilic inflammation --> bronchiectasis
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