Term
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Definition
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Term
When does the respiratory system begin to develop? |
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Definition
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Term
What structures are part of the conducting zone? |
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Definition
nasal cavity, pharynx, larynx, trachea, bronchi, bronchioles |
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Term
What structures are part of the respiratory zone? |
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Definition
respiratory bronchioles, alveolar ducts, alveolar sacs and the alveoli |
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Term
What structures are cephalic to the larynx? |
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Definition
nasal and oral cavities, pharynx |
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Term
The formation of the lung bud is caused by what process? |
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Definition
lateral folding of two sphlanchnic mesoderm and endoderm that meed at midline to separate the larynx and trachea from the esophagus |
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Term
The larynx is first seen as what structure? |
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Definition
ventral outgrowth of endoderm from the foregut --> lung bud/respiratory diverticulum |
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Term
The larynx arises from what early embryology tissues? |
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Definition
1. epithelium: endoderm 2. muscles and cartilage: neural crest cells |
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Term
The trachea develops from what tissues? |
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Definition
1. epithelium: endoderm 2. muscles/cartilage comes from splanchnic mesoderm |
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Term
At the end of lung development in early childhood, how many divisions have the bronchi undergone? |
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Definition
24 generations of branching |
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Term
List, in order of earliest to latest in development, the branching of the trachea to form bronchioles. |
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Definition
Trachea --> L/R bronchi --> R bronchus gives off 3 diverticula and L bronchus gives off two diverticula ---> lobular bronchi --> smaller bronchi ---> bronchioles |
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Term
At birth in a term baby, how many generations of branching has occurred? |
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Definition
more than 17, but less than 24 |
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Term
Name the 4 stages of development for the lungs |
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Definition
Pseudoglangular, canclicular, terminal sac, and aveolar period |
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Term
What happens during the pseudoglandular period? |
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Definition
-bronchi are dividing into bronchioles. Typically from week 5th-28th week -trachea and foregut separate |
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Term
What is the canalicular period |
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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 |
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Term
Describe the events of the terminal sac phase of lung development |
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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 |
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Term
What cells produce surfactant? |
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Definition
type II alvelolar epithelial cells? |
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Term
At what stage in lung development does the fetus become viable? Why? |
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Definition
during the terminal sac phase - weeks 25/26 and on because type I alveolar cell epithelial cells are capable of gaseous exchange |
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Term
What occurs during the alveolar period and when does it happen during development? |
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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 |
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Term
What is the septum transversum and how does it develop? |
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Definition
-separates the throacic cavity and abdominal cavity -develops from body wall after lateral body folding is completed |
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Term
What are the pericardioperitoneal canals? |
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Definition
communication between the abdominal and thoracic cavity that remains |
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Term
Explain the embryologic developments that occur to result in on peridcardial cavity and two pleural cavities |
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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 |
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Term
The diaphargm develops from which four structures? |
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Definition
1. lateral body wall mesoderm 2. septum transversum 3. dorsal mesentery of the esophagus 4. pleuroperitoneal folds |
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Term
Explain the development of the diaphragm |
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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 |
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Term
Where does the phrenic nerve found? |
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Definition
embedded in the fibrous pericardium as it travels with the diaphragm during development |
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Term
What is the diaphragm innerveated by? |
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Definition
3-5th cervical spinal cord segments via phrenic nerve |
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Term
What is the earliest lung malformation? |
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Definition
Agenesis fom fialure of two bronchial buds to form from the larynx (lung bud) |
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Term
What condition results from an errorrs of development during the pseudoglandular period? |
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Definition
-TEF or esophageal atresia -Congenital diaphragmatic hernia & lung hypoplasia -abnormal bronchial divison disorders (bronchogenic cysts & pulmonary sequestration) |
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Term
What is the most common type of TEF? |
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Definition
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Term
What is congenital diaphragmatic hernia? |
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Definition
herniation of abdominal contents into the chest thorax preventing lung development |
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Term
Why is CDH most commonly left-sided? |
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Definition
nothing preventing herniation - liver on R side |
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Term
What are the assessment findings associated with CDH? |
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Definition
scaphoid abdomen and respiratory distress -usually dx prenatally |
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Term
Why do we give corticosteriods to preterm babies? |
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Definition
1. cuboidal --> squamous conversion 2. terminal sac development 3. increase in # of bronchial divisions 4. vasculature development (more capillaries - alveoli connections) |
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Term
Why are steroid induced growth factors necessary? |
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Definition
to facilitate bronchial divisions, vasculature, epithelium, CT development |
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Term
What congenital condition results from an error in development during the alveolar period? |
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Definition
Pulmonary hypoplasia (NOT agenesis) |
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Term
What is pulmonary hypoplasia? |
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Definition
-reduced number of bronchioles, terminal bronchioles, alveoli, and arterioles -often assoc. with Tris 18, 21 |
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Term
What causes pulmonary hypoplasia? |
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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) |
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Term
At birth, infants have how many alveoli? |
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Definition
50 million (~5% of total) |
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Term
By what age does alveolar development stop? |
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Definition
8 years old. 300million alveloi (~95%) |
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Term
What three disorders result from abnormal bronchial divisions? |
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Definition
1. bronchogenic cyst 2. pulmonary paranchymal cyst 3. pulmonary sequestration |
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Term
What are bronchogenic cysts and what is the treatment prescribed? |
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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 |
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Term
What is pulmonary paranchymal cyst? |
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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 |
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Term
What is pulmonary sequestration? |
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Definition
-abonromal, accessory bud that retains arterial connections with GI vasculature ==> non fuctional, ectopic lung tissue with no blood supply ==> no gas exchange occurs |
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Term
What are the different types of pulmonary sequestration? |
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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 |
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Term
How is pulmonary sequestration dx and what is the treatment? |
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Definition
-MRI/MRA and angiography for Dx -surgical resection - lobectomy or resection en bloc (extralobar sequestration) |
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Term
What is Cystic adnomatoid malformation (CAM or C-CAM) |
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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 |
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Term
What is congenital lobar emphysema (CLE)? |
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Definition
overdistention and airtrapping of a lung lobe due to a ball-valve like mechanism -more common in males, 20% are bilateral, fairly uncommon |
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Term
What are the common causes of CLE? |
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Definition
-deficiency of cartilate -enlarged vascular structure -overlying mass or intrathroacic mass from lymphadenopathy or bronchogenic cyst |
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Term
What is the role of surfactant in preventing atalectasis? |
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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 |
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Term
What is surfactant made of? |
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Definition
phospholipids and proteins |
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Term
What stimulates surfactant production? |
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Definition
stretching of the air spaces with berathing |
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Term
During what period do fetal breathing movements occur? |
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Definition
alveolar period. Breathin movements condition respiratory muscles and stimulates alveolar development/growth |
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Term
What is congenital alveolar proteinosis? |
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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 |
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Term
Explain meconium aspiration and the neonate |
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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 |
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Term
What is persistent pulmonary hypertension of the newborn and why does it occur? |
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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 |
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Term
Why does hypoxemia result from PPHN? |
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Definition
decreased pulmonary perfusion because of high PVR |
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Term
What are the long-term effects of persistent pulmonary HTN? |
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Definition
1. hypoxemia 2. decreased cardiac output if no cardic shunt exists 3. cor pulmonale |
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Term
What are the treatment options for pulmonary hypertension? |
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Definition
1. correct lesion or underlying disease 2. promote pulmonary artery vasodilation (NO/prostacyclins) 3. increase myocardial contraction 4. diuresis |
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Term
What is PDA and why does it occur? |
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Definition
-when closure of DA fails to take place because of high pulmonary vascular resistance |
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Term
What are the effects of PDA and PFO? |
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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 |
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Term
What are the characteristics of lung diesease of prematurity? |
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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 |
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Term
What is bronchopulmonary dysplasia? |
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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 |
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Term
What is the treatment of Lung disease of prematurity? |
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Definition
-Surfactant instillation -mecahnical ventilation and supplemental oxygen -bronchodilators -steroids -diurectics -long term ventilation via trach bc of chronic respiratory failure |
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Term
Define: ventilation, gas exchange, oxygenation, transport, and regulation. |
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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 |
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Term
What structures belong to the extra-thoracic part of the conducting zone? The intra-thoracic? |
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Definition
Extra-thoracic: -nose/mouth, trachea, larynx, pharynx Intra-thoracic: -bronchi & bronchioles |
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Term
What is airway resistence and how is it determined? |
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Definition
-determined by diameter of the conducting zone structures. - R= 1/r^4 |
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Term
Describe the mucous lining and cilia characteristics of of the nose --> terminal bronchioles |
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Definition
1. globet cells that secrete mucous 2. ciliated epithelium with cilia that constantly beat |
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Term
Where is the "upper airways" and what structures are inclded? |
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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 |
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Term
What are important considerations regarding the upper airways? |
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Definition
-must remain patent -must have functioning ciliated epithelium and produce mucous |
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Term
What is the purpose of the upper airways? |
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Definition
-move air into the lungs -humidifying an cleaning debris from air entering the lungs |
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Term
What are the developmental differences in the upper airways between a child and an adult? |
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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) |
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Term
Name the common nasal disorders. |
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Definition
-Infectious Rhinitis -allergic rhinitis (acute/chronic) -nasal polyps (CF) -sinusitis (may be associated with Kartagener Syndrome) |
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Term
What is olfactory neuroblastomas? |
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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 |
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Term
What are the common assessment findings of nasopharyngitis, tonsilitis, and laryngitis? |
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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) |
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Term
What causes nasopharyngitis, tonsilitis, and laryngitis? |
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Definition
-usually viral in origin (Rhinovirus, echovirus, adenoviruses, RSV, influenza) -bacterial infections may be superimposed on viral |
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Term
What is stridor and what causes it? |
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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 |
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Term
What Is are the common assessment findings of peritonsillar abscess? |
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Definition
-muffled voice -drooling -stridor -trismus -will have jaw movement |
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Term
When do peritonsillar abscesses typically form? |
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Definition
early in the second decade of life (early teens) |
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Term
What causes peritonsillar abscess? |
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Definition
most often bacterial in origin -staph aureus, micrococcus aureus, beta-hemolytic strep and anaerobic bacteria |
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Term
Compare/contrast pertonsillar and retropharyngeal abscess |
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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 |
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Term
What does retropharyngeal abscess look like on an Xray image? |
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Definition
prominent soft tissue swelling of the posterior pharynx. Much larger space than normal |
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Term
What is a primary concern in treating someone with epiglottitis? |
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Definition
Airway obstruction --> EMERGENCY |
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Term
What causes epiglottitis? |
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Definition
haemophilus influenza B (vaccine), bacterial infection, Candida -between 2-7yrs |
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Term
What are the s/s of epiglottitis? |
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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. |
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Term
What is laryngeal papillomatosis and what causes this condition? |
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Definition
-perinatal infection with HPV -onset: infancy - 4 years |
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Term
What is the most common congenital anomaly of the airway? |
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Definition
laryngomalacia and tracheomalasia |
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Term
What are some common s/s of laryngomalacia & tracheomalasia |
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Definition
-inspiratory stridor that worsens with crying -hypercapnea (hypoventilation) -bronchoscopy:arytenoid cartilages and all supraglottic structures collapse inward (toward glottis) |
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Term
What is laryngomalacia/tracheomalasia? |
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Definition
Trachea rings not strong enough to maintain patent airway during inhalation |
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Term
What is a laryngeal web and what is the recommended treatment? |
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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 |
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Term
Describe the vocal cord movement during inspiration/expiration |
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Definition
-cords are open during inspiration/phonation -cords are closed during exhalation and at rest |
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Term
Where are the vocal cords located? |
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Definition
protrude from the lateral walls of the glottis and are stretched and posistied by muscles, ligaments, and cartilage |
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Term
What happens to the vocal cords to during phonation? |
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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 |
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Term
Describe vocal cord paralysis |
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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 |
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Term
What condition results from upper airway trauma. |
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Definition
-compression or direct trauma on tracheal mucosa cuases edema, necrosis, and granuloma formation -induces subltottic stenosis, laryngotracheomalacia, vocal cord injury |
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Term
What is a common example of upper airway trauma? |
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Definition
post-extubation extrathoracic airway obstruction - 35% |
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Term
What is laryngotracheobronchitis? |
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Definition
-also known as Croup -often occurs seasonally in the winter in 3mos to 3years |
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Term
What are the symptoms of croup? |
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Definition
-gradual onset - low grade fever - bark-like cough -inspiratory stridor -no sore throat/normal voice/no drooling, non-toxic appearance |
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Term
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Definition
virus - parainfluenza virus A, adenovirus, RSV |
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Term
What is the classic sign of croup on an xray? |
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Definition
"steeple sign" with narrowing of traceal air column at the larynx and distension at the hypopharynx |
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Term
What is tracheitis and when does it occur? |
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Definition
-infection/inflammation of the trachea |
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Term
What are the symptoms of tracheitis? |
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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 |
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Term
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Definition
high pitched whislting sounds on exhalation due to obstruction in intrathoracic airways |
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Term
Compare the extra-thoracic airway vs th intra-thoracic airway |
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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 |
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Term
Describe the difference in cartilage formation in the trachea, bronchi, and bronchioles |
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Definition
Trachea: cartilage rings Bronchi: cartilage plates Bronchioloes: no cartilage and depend on transpulmonary pressures to remain open |
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Term
Describe the smooth muscle structure in bronchi and bronchioles |
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Definition
Bronchi: -smooth muscles in areas wihtout cartilage plates Bronchioles -almost exclusively made of smooth muscle |
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Term
Describe the innervaiton of bronchi and bronchioles |
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Definition
-sympathetic nerve fibers that act on beta 2 receptors -parasympathetic nerve fibers of vagus nerve |
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Term
What is the effect of epinephrine on the bronchi/bronchioles |
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Definition
acts on beta-2 receptors to cause vasodilation |
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Term
Vasoconstriction in bronchial smooth muscle is caused by what substances? |
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Definition
acetylcholine, leukotriences (phosphase lipase A & arachidonic acid metabolism), histamine (anaphylaxis) |
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Term
What are the different disorders of the intrathroacic conducting zone airways? |
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Definition
bronchitis, bronchiolitis, asthma, bronchiectasis |
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Term
What is bronchitis and what is it caused by? |
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Definition
-inflammation of the bronchi -Cause: viral or bacterial infection; may be allergic or irritant -typically non-obstructive and produces exudate |
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Term
What is bronchiolitis and how is it caused? |
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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 |
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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 |
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|
Term
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Definition
allergic, hypersensitive, inflammatory and obstructive lung disease *bronchial constriction with swelling and mucous production |
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Term
What immunglobulin mediates asthma reactions? |
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Definition
IgE mediated inflammation: arachidonic acid, leukotrienes, acetylcholine, histamine, prostaglandin D2, platelet aggregating factor, various cytokines |
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Term
What are the effects of asthma? |
|
Definition
-air trapping/hyperinflation with adjacent atalectasis -hypercarbia -hypoxemia when gas exchange is impaired |
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Term
What is the long-term effect of chronic asthma? |
|
Definition
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|
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 |
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Term
Describe the process of airway remodeling in asthma |
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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 |
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Term
What are the pharmacologic treatments for asthma? |
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Definition
Bronchodilators and anti-inflammatory agents |
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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 |
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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) |
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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 |
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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 |
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|
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 |
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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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