Pathophysiology Flashcards
Cystic fibrosis (CF) is an ____ disease.
Autosomal recessive
CF is caused by mutations in the…
CF transmembrane conductance regulator gene (CFTR)
CFTR regulates…
Chloride ion movement across apical membranes
Dysregulation of CFTR results in disruption of ____, therefore impact composition of…
Chloride secretion, sodium reabsorption, water transport - impact composition of secretions and mucus to be thick
…Causing manifestations of CF
Thick and sticky secretions in CF patients result in…
Obstruction of ducts on various organs, and small airways on the lungs
Chronic infection +/- inflammation in many different areas of the body
Thick lung secretions lead to…
Bacterial colonization
Pulmonary symptoms involved in CF include…
Chronic, persistent productive cough that may include pus/blood in sputum
Dyspnea, wheezing, chest pain
A pulmonary exacerbation is…
An increase in respiratory symptoms, with introduction of systemic symptoms
Such as malaise, anorexia, weight loss
Routine ____ are done to track pulmonary function
PFT’s
Thick secretion in the GIT can manifest as…
GERD, constipation
Intestinal obstruction, bacterial overgrowth, rectal prolapse
These vitamins have decreased absorption in CF…
Fat-soluble vitamins
Pancreatic insufficiency in CF is caused by…
Decreased volume of pancreatic secretions in ducts - causing destruction of pancreas since secretions are retained
Pancreatic insufficiency results in…
Decreased absorption of lipids + fat-soluble vitamins
Long-term complications: chronic pancreatitis, glucose intolerance, CF-related diabetes
Infertility is a likely CF complication due to…
Congenital absence of vas deferens in males
Thick cervical mucus in females
Goals of therapy for CF include…
Maintain lung function as much as possible, ongoing
Maintain adequate nutrition, ongoing.
Maintain overall QoL
