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