HLTH 2501: obstructive lung diseases Flashcards
cystic fibrosis
is an inherited disorder that involves several mutations to the CFTR and also a protein involved in chloride ion transport into the cell membrane
CFTR
cystic fibrosis transmembrane conductance regulator
major effect of cystic fibrosis
exocrine glands cause abnormally thick secretions often obstructing passageways in the lungs and pancreas
obstruction in the lungs due to cystic fibrosis
mucus obstructs the airflow in the bronchioles, causing air trapping or atelectasis; infections are also common here due to the stagnant mucus that is good for bacterial growth
common causative organisms for secondary infections in cystic fibrosis
P aeruginosa and S aureus
what will eventually develop with cystic fibrosis?
respiratory failure or right-sided CHF
cystic fibrosis in the digestive tract
in infants, the small intestine is blocked by mucus at birth; in the pancreas and liver, the ducts of the exocrine glands become blocked, leading to a deficit of pancreatic enzymes and bile in the intestine, resulting in malabsorption and malnutrition; damage to the islets of Langerhans may also occur, leading to diabetes mellitus
meconium ileus
occurs in newborns with cystic fibrosis, in which the small intestine is blocked by mucus
general effects of cystic fibrosis in the digestive tract
malabsorption, malnutrition, and dehydration
salivary glands and cystic fibrosis
are mildly affected, with secretions that are abnormally high in NaCl and mucus plugs in the submaxillary and sublingual glands
sweat glands and cystic fibrosis
they produce sweat high in NaCl content
what can obstruction of bile ducts in the liver result in?
biliary cirrhosis
reproductive system and cystic fibrosis
thick mucus may obstruct the vas deferens in males or the cervix in females, leading to sterility or infertility
genetical inheritance for cystic fibrosis
the mutated gene is CFTR and is located on the seventh chromosome and is transmitted as an autosomal recessive disorder
signs of cystic fibrosis
meconium ileus at birth, salty skin, steatorrhea, distended abdomen, failure to gain weight, chronic cough, common respiratory infections, and failure to meet normal growth milestones
testing for cystic fibrosis
can be done through genetic testing to identify the CFTR mutations at birth, sweat or stool can also be analyzed, as well as X-rays, pulmonary function tests, and blood gas analysis
treatment for cystic fibrosis
replacement of pancreatic enzymes and bile salts, a well balanced diet (high calorie, high protein, low fat, and vitamin supplementation), avoiding dehydration, intensive chest PT, bronchodilators, humidifiers, and regular moderate aerobic exercise
example of a pancreatic enzyme replacement drug
pancrelipase
what is often the cause of death for cystic fibrosis?
respiratory failure
lung cancer
is the third most common cancer in the US and is most often malignant tumors that are primary or secondary
why are the lungs a common site for secondary tumors?
because the venous return and lymphatics bring tumor cells from many distant sites in the body to the heart and then into the pulmonary circulation
two main groups of lung cancers
small cell lung cancer (13%) and nonsmall cell lung cancer (84%); differences between these groups are cellular morphology, rate of metastasis, and treatment
small cell lung cancer
the cells are smaller and round, spread rapidly, and have a higher mortality rate; often is associated with smoking and is resistant to chemo
nonsmall cell lung cancer
the cells are larger, the spread is less aggressive, and responds well to chemo
subtypes of nonsmall cell lung cancer
adenocarcinoma, squamous cell carcinoma, and large cell carcinoma
the most common type of lung tumor
bronchogenic carcinoma; this arises from the bronchial epithelium
squamous cell carcinoma
usually develops in the epithelial lining of a bronchus near the hilum and projects into the airway
adenocarcinomas
are from glands that secrete mucin and are found on the periphery of the lung, making them harder to detect in the early stages
oat cell carcinoma
are located near a major bronchus in the central part of the lung; tend to be invasive and metastasize early
mesothelioma
has received attention from legal firms as it may be caused by asbestos exposure
pleural mesothelioma
most often affects the pleura surrounding the lungs; usually is fatal
first change in the lungs for lung cancer
is metaplasia which is a change in the epithelial tissue often associated with smoking or chronic irritation, leaving the tissue vulnerable to irritants and inflammation
second change in the lungs for lung cancer
dysplasia or carcinoma in situ develops
common site for tumors spreading from the lungs
brain, bone, and liver
effects of tumors in the lungs
obstruction of airway flow causing abnormal breath sounds and dyspnea, inflammation that causes a cough, vulnerability to secondary infections, pleural effusion, paraneoplastic syndrome, and systemic effects of cancer
paraneoplastic syndrome
may accompany bronchogenic carcinoma when the tumor cell secretes hormones or hormone like substances such as ADH or adrenocorticotropic hormone
what do tumors on the periphery of the lung often result in?
hemothorax (blood in the pleural space), pneumothorax (air in the pleural cavity), and pleural effusion (fluid in the pleural cavity)
risk factors for developing lung cancer
smoking, second hand smoke, genetic factors, chronic obstructive pulmonary disease, exposure to carcinogens such as silica, vinyl chloride, or asbestos
4 possible categories of signs of lung cancer
those related to the direct effects of tumor on the respiratory structures, those representing the systemic effects of cancer, those caused by associated paraneoplastic syndromes, and those resulting from metastatic tumors at other sites
early signs related to respiratory involvement of lung cancer
persistent cough, dyspnea, wheezing, hemoptysis (coughing up blood), pleural effusion, pneumothorax, hemothorax, chest pain, hoarseness, facial or arm edema, headache, or atelectasis
systemic signs of lung cancer
weight loss, anemia, and fatigue
paraneoplastic syndrome signs of lung cancer
is indicated by the signs of an endocrine disorder related to the specific hormone secreted
signs of a metastasis of lung cancer
specific to the site; ex. bone spread can cause bone pain or a pathologic fracture