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Clinical Medicine Cystic Fibrosis Month 1 Week 1 Day 1
Clinical Medicine Cystic Fibrosis Month 1 Week 1 Day 1
28
Medical
Graduate
05/07/2018

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Term
inheritance of cystic fibrosis
Definition
autosomal recessive, if each parent is a carrier one child will have it, one child won't, and 2 will be carriers
Term
abnormal gene in CF
Definition
CFTR, cystic fibrosis transmembrane conductance regulator, encoded by a gene on chromosome 7, primarily affects cyclic AMP-regulated chloride channels, mutations in both copies of the gene results in clinical disease
Term
other channels affected by cyclic AMP-regulated chloride channels
Definition
sodium bicarb and water transport
Term
What happens with the CF mutation
Definition
get premature transcription termination and then no protein is expressed or there is protein misfolding for the cl channel protien
Term
most common CF mutation
Definition
deletion of F508
Term
what happens in CF disease
Definition
one side of the cell has normal cl channels and the other does not so more cl into cell but can't move it out so get sticky mucus building up on outside of cell (leads to pulmonary and pancreatic changes, inc secretions in lungs) (mucus traps bacteria leading to respiratory infections) (get inc'd water absorption due to defective channel) bacterial infections often pseudomonas or staph aureus. recurrent infections lead to recurrent inflammation and destruction of pulmonary airways leading to bronchiectasis and lung insufficiency, takes years to occur
Term
clinical manifestations of CF
Definition
multisystem- sinuses, lungs, pancreatic disease, liver disease, intestines, male reproductive tract (can't make sperm), infertility in females (less commonly) bone involvement- osteoporosis, cystic fibrosis related diabetes, kidney stones, premature cardiovascular disease, GI cancer
Term
what happens in the lungs in CF
Definition
dehydration of the respiratory epithelium and defective mucus clearance results in this increase in viscous secretions predisposing the pt to bacterial colonization leading to bronchioectasis, atelectasis, collapsing of the lungs, recurrent pneumonias, progressive airway obstruction, death from respiratory failure
Term
bronchioectasis
Definition
continued widening, scaring and damaging of the airways
Term
recurrent infection and inflammation of lungs leads to
Definition
bronchial cysts which get hypertrophied, then get brachial circulation, pulmonary hypertension and respiratory failute
Term
CF organisms in childhood
Definition
staph aureus and haemophilus influenza
Term
CF organisms in adults
Definition
, pseudomonas aeruginosa, burkholderia cepacia, mycobacterium avium, aspergillus
Term
diagnostic clinical features of CF
Definition
cough, sputum production, wheezing, air trapping, chronic sinus disease, meconium ileus at time of delivery, pancreatic insufficiency, current pancreatitis
Term
diagnostic testing for CF
Definition
sweat chloride (>60 mmol/L x2 is pos) (higher in older adults), genetic testing for mutation (CFTR mutation analysis)
Term
why is it impt to diagnose CF early by age 3
Definition
if not then a third of the children at age 3 will have mucus obstruction, bronchioectasis, and inflammation, lung damage
Term
other things that can cause an elevated sweat chloride
Definition
malnutrition, untreated adrenal insufficiency, untreated hypothyroidism, but also anorexia, atopic dermatitis, G6PD deficiency, some of the glycogen storage diseases
Term
CF treatment
Definition
reducing the increased secretions, airway clearing, postural drainage, percussion, direct cough, Thairapy vest, exercise (inc FEC and FEV1), hypertonic saline (be careful 1st time)
Term
anti-inflammatory treatments for CF
Definition
chronic systemic steroids (slow down decline in lung function but SEs), inhaled corticosteroids, chronic NSAIDS in 5-12 year olds
Term
antibiotics for CF
Definition
azithromycin (also anti-inflammatory), macrolides (standard of care) (anti-inflammatory and anti-infective) (give long term for pseudomonus) (can break down the biofilm pseudomonas forms on surface of lungs), chronic nebulized antibiotics- tobramycin, aztreonam, colistin
Term
SE of colistin
Definition
broncho reactivity, spasms, sulfur smell
Term
type of respiratory failure in CF
Definition
hypoxemic (put on supplemental O2), hypercapnic (assisted ventilation of BiPAP)
Term
for hemoptysis in CF
Definition
control the breathing, control airway
Term
CF patient are often deficient in
Definition
vit K
Term
treatment for osteoporosis
Definition
vit D, calcium, exercise, possibly bisphosphonates
Term
test for pancreatic abnormalities
Definition
stool fecal fat
Term
treatment for pancreatic issues
Definition
fat soluble vitamins, pancreatic enzyme replacements(lipase, proteases, amylase- extracts from porcine or pig pancreases, take with each meal), high caloric intake
Term
CF related diabetes treatment
Definition
insulin (due to fibrosis in pancreas)
Term
what to monitor in CF patients, how to treat
Definition
head circumference, height, weight, length, BMI, height goal, 24 hr diet recall, lung function, infections, albuterol to open airways, chest physiotherapy to loosen secretions, hyptertonic saline, inhaled antibiotics, exercise, fat soluble vitamins, high calorie supplements, watch for comorbid conditions
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