Neonatal, Developmental, and Vascular Pulmonary Disease--Handorf Flashcards
What is the secondary function of the lung?
protect us from airborne toxins… via
- upper airway filter (nose)
- Tracheobronchial mucociliary apparatus
- Intraalveolar immune/phagocytic system
Which bronchus is more vertical? What is the significance of this?
right is more vertical = right lung is more likely to get aspiration pneumonia (shit can get in there easier)
What are pores of Kohn? What is the significance of them?
holes in the alveoli that connect adjacent alveoli = they function as a means of collateral ventilation
*that is, if the lung is partially deflated, ventilation can occur to some extent through these pores
**also fluid and microbes can spread through these holes = more lung affected
Where are cancer mets most likely to form in the lung first? Why?
hematogenous spread = at the base bc the perfusion is better there
Where in the lung is TB likely to appear? Why?
upper lobed = more air there which they need for growth
If part of the lung is infarcted (PE), will it be a red or white infarct? Why?
red bc the lung has a dual blood supply
Why does a diaphragmatic hernia often lead to pulmonary agenesis or hypoplasia?
The hernia allows for abdominal contents to move into the thoracic cavity during development –> there is less space in the thoracic cavity –> lungs cannot develop to the correct size bc they do not have enough room
What conditions can lead to pulmonary agenesis or hypoplasia?
- diaphragmatic hernia
- renal cysts
- atresia (condition in which a body orifice or passage in the body is abnormally closed or absent)
- right heart abnormalities
- pulmonary sequestration
- musculoskeletal disorders ??? not talked about in robbins?
What are the tracheobroncial abnormalities? (none of which are in any of our textbooks so I’m not going to explain them)
common laryngotracheoesophageal tract
tracheoesophageal fistula
tracheal atresia
tracheobronchomalacia
What are common problems/symptoms seen with tracheobronchial anomalies?
recurrent atelectasis of the right upper lobe (RUL) recurrent pneumonia congenital stridor (fancy term for noisy breathing due to partial airway obstruction)
**ripped form NCBI article
What is the most common type of tracheoesophageal fistula?
one in which the trachea communicates with the distal segment of an atretic esophagus
(87%)
What problems/symptoms/signs will you see with a tracheoesophageal fistula
coughing with feeding spells of cyanosis GI tract distended with air pneumonia/RUL opacity on CXR aspiration fever, tachycardia, respiratory distress
What is congenital lobar overinflation?
Pathophys?
What lung or parts of the lung does it typically affect?
Symptoms?
emphysema in infancy = air cannot leave the lungs
due to cartilage deficiency in distal bronchial tree –> airway obstruction
50% ULL and 25% RML
SOB, wheezing, cyanotic lips and nail beds
Congenital (bronchogenic) Cyst:
Epidemialogy?
Pathology?
How is it diagnosed?
- -age 14-30
- -fragment of foregut filled with mucous (bronchial epithelium without attachment to tracheobroncial tree) that can be found anywhere in lungs, mediastinum, pericardium, or diaphragm
- -routine CXR, infection occurs, or when it ruptures
What is pulmonary sequestration?
What is it assc with?
an “accessory lung” is formed when lung tissue forms within (intralobar) or outside (extralobar) normal lung tissue but there is no airway connect to the tracheobronchial tree. it also usually has an independent arterial supply directly from the aorta or the pulmonary artery
assc with reccurnet local infection, and extralobar causes a mass lesion to form
What are the aspiration syndromes
- inhalation of amoniotic fluid in utero due to fetal distress
- inhalation of bloody amniotic fluid peripartum
- inhalation of gastric contents postpartum
Microscopically, what is seen in lung tissue with meconium aspiration?
brown globules (poop)
flaky squamous epithelial cells
**both in alveoli
What are the 3 types of atelectasis and name what causes each (general causes)?
- resorption: obstruction of airway prevents inflation
- compression: mass (fluid, air, etc) within the pleural cavity compresses and collapses the lung
- contraction: internal fibrosis
What type of atelectasis will CHF cause?
pneumothorax?
compression: pleural effusion
compression? air
What kind of atelectasis is NOT reversible?
contraction
What are common complications of atelectasis?
infection of collapsed lung
hypoxemia (more like a consequence)
What type of atelectasis is hyaline membrane disease assc with?
resporptive
Hyaline membrane disease:
Pathophys?
Pathology?
epidemiology?
physicochemical “shock” of lung expansion at birth
Pulmonary edema –> membrane formation –> resorptive atelectasis –> bronchiolar epithelium necrosis
Male predominance, premature neonates, mild maternal diabetes, C-section delivery, seasonality (late autumn/early winter)
What is pulmonary edema and what are the 2 causes of it (general)
Intraalveolar pooling of proteinaceous fluid due to (1) hemodynamic causes or to (2) microvascular injury
What are the hemodynamic causes of pulm edema?
increased hydrostatic pressure
–left sided heart failure, mitral stenosis, volume overload, pulmonary vein obstruction
Decreased oncotic pressure
–hypoalbuminemia, nephrotic syndrome, liver disease
What are the microvascular causes of pulm edema?
Infectious agents irritant gase toxic drugs shock/trauma/sepsis radiation
Describe the pathophysiology of ARDS
Diffuse capillary wall insult –> IL-8, IL-1, TNF released –> edothelial activation and neutrophil recruitment and activation –> neutrophils release PAF, LTs, and proteases that damage alvolar epi- and endo-thelium –> Increased capillary permeability –> Interstitial/intraalveolar edema –> surfactant dysfunction (due to damaged type II pneumocytes) –> -> Hyaline membrane formation –> release of macrophage derived PDGF and TGF-Beta –> Fibrin exudation/deposition (healing phase)
- ->Death (20%) or resolution with scarring
- ->Sepsis
- ->Pulmonary infections
What are possible etiologies of ARDS?
1) physical injury: burn, near downing, mechanical trauma
2) inhaled irritants: smoke, gases
3) chemical injury: aspirin, paraquat
4) hematological condiations
5) pancreatitis
6) uremia
7) cardiopulmonary bypass
Describe the usual clinical course of ARDS
rapid onset of life-threatening respiratory insufficiency, cyanosis, and severe arterial hypoxemia that is refractory to oxygen therapy and may progress to multisystem organ failure
What s the histological manifestations of ARDS?
diffuse alveolar damage (DAD)
-capillary congestion
-necrosis of alveolar epithelial cells
interstitial and intra-alveolar edema and hemorrhage
-collections of neutrophils in capillaries
–> hyaline deposits –> thickening of alveolar septa = resolution/resorption of hyaline membranes
How is ARDS in the newborn distinctly different from ARDS in an adult?
in the newborn, it is due to a surfactant deficiency
What are the 2 pulomonary hemorrage syndromes?
goodpastures
granulomatosis wih polyangiits
What is the etiology of goodpastures?
epidemiology?
antibodiesto thebasement membranes (with specificity against the noncollagenous domain of the alpha-3 chain of collagen 4) cause glomerulonephrits and lung hemorrages
young makes in 20-30s
What is granulomatosis with polyangittis?
autoimmune disease affecting lung and URT (may affect kidneys) –> perivascular granulomas form = systemic vasculitis
**c-ANCA Abs (specifically anti-PR3 proteinase antibodies) cause the necrotizing vascualitis –> pulm hemorrhage
