Lecture 6 - Respiratory Pathologies: Congenital, Parenchymal, Vascular, and Pleural Disorders Flashcards
cystic fibrosis is a hereditary disorder affect what glands?
exocrine (decreased Na and Cl ion transport)
cystic fibrosis causes production of abnormally thick mucus which leads to blockages of the lumen of what 3 areas?
pancreatic ducts, intestines and bronchi (respiratory infections)
cystic fibrosis leads to what other condition?
COPD (and progressive loss of pulmonary function)
what is the most common autosomal-recessive (both parents are carriers) fatal disease?
cystic fibrosis
due to the blockage of the pancreatic duct, digestive enzymes are affected, resulting in malabsorption that leads to what?
retarded growth
what is the most important complication of cystic fibrosis?
viscous bronchial mucus plugs preventing normal breathing
what are complications of cystic fibrosis, besides viscous bronchial mucus plugs?
mucus = bacterial growth chronic bronchitis recurent pneumonia bronchiectasis pulmonary fibrosis
define ‘atelectasis’
partial or complete collapse of lung
what is the primary cause of atelectasis, and why?
bronchial obstruction because air in the alveoli is absorbed into blood and alveoli collapse
what are other causes of atelectasis besides bronchial obstruction?
failure to breath deep enough insufficient surfactant (premature babies) direct compression (pnuemothorax) pleural effusion/inflammation smoke inhalation
what are 6 acute clinical manifestations of atelectasis?
dyspnea tachypnea cyanosis fever low BP shock
what are 2 chronic clinical manifestations of atelectasis?
gradual onset of:
dyspnea and weakness
(possible to be asymptomatic)
define ‘pulmonary edema’
accumulation of excessive fluid in the interstitial tissue, alveoli or both
name 5 conditions that pulmonary edema is a common complication of:
left sided heart failure water retention conditions (kidney disease) smoke inhalation shock acute respiratory distress syndrome
how does pulmonary edema manifest in initial stages?
possibly asymptomatic or could shows signs of restlessness, anxiety or feel-like developing a cold
how does pulmonary edema manifest as fluid builds up?
progressive dyspnea persistent cough with frothy sputum and hemoptysis exercise intolerance tachypnea wheezing hypoxia/cyanosis/hypercarpnia/acidosis
T or F: pulmonary edemas prognosis is often reversible, depending on the underlying condition?
true
with acute respiratory failure, lungs will show what?
DAD - diffuse alveolar damage
what are 6 possible causes of acute respiratory distress syndrome?
severe trauma pulmonary infection/aspiration prolonged cardiopulmonary bypass shock fat emboli sepsis
when does ARDS occur?
within 12-48 hours after initiating event
what are 6 SSx of ARDS?
shallow, rapid breathing dyspnea pulmonary edema atelectasis chest retractions cyanosis
what does MODS stand for?
multiple organs dysfunction syndrome
what are 4 ways of treating ARDS?
treat underlying condition
CPAP machine
mechanical ventilation (PEEP)
sedations
what does PEEP stand for?
positive end expiratory pressure
what % of survivors from ARDS experience lung fibrosis?
40%
what fraction of people die from ARDS within days?
1/3 - often from pneumonia
define ‘sarcoidosis’
growth of tiny collections of inflammatory cells in different parts of lungs due to an unknown cause
what organs can sarcoidosis affect?
lungs (90%) lymph nodes of thorax and neck liver lacrimal and salivary glands (1/3) eyes skin
what is a theory for the cause of sarcoidosis?
maybe an immune response to an unknown substance, most likely inhaled from air
is there a cure for sarcoidosis?
there is no cure, but most do well from modest treatment and it often goes away on its own (70% recover spontaneously within a year or 2, 20% persist and 10% lethal)
define ‘pneumoconioses’
lung disease caused by inhalation of mineral dust, organic and inorganic particles (occupational disease - coal workers, silicosis, asbestosis)
how do lungs present with coal-worker’s lung disease?
black, fibrotic and structurally abnormal
what is the common name for antracosis?
coal-workers lung disease (develops in substandard work conditions)
what condition does coal-workers lung disease predispose you to?
TB (not lung cancer)
what is the most common lung disease caused by mineral particles from the environment?
silicosis
what causes silicosis?
inhalation of small silica crystals from dust generated from - stone cutting, mining, sand blasting (develops 10-20 years after exposure)
what cell does silica damage or kill?
macrophages
what mechanism of silicosis causes massive fibrosis?
dead macrophages release free silica particles and fibrogenic factors that stimulates fibroblasts and produces collagen leading to formation of collagenous nodules, eventually causing silicotic nodules that destroy the parenchyma of the lungs leading to scaring/ fibrosis of lungs
what is a common complication of silicosis?
TB (not lung cancer)
what are 4 conditions linked to asbestos exposure?
pulmonary fibrosis
pleural fibrosis
lung cancer
mesothelioma
true or false: asbestos kill macrophages?
false (macrophages release substances that result in extensive pulmonary fibrosis)
how much more frequently do malignant tumors develop in fibrotic lungs?
5-6 times more frequently
when smoking is combined with asbestosis how much higher is the risk of lung cancer?
50 times higher
what pathology occurs almost exclusively in people exposed to asbestos?
mesothelioma
