Answers
cystic firbrosis exocrine gland (mucous producing) dysfunction; causing thick mucous to
mechanical obstruction of organs
which organs are affected by CF? lungs
pancreas
liver
small intestine
reproductive organs
, cystic fibrosis transmembrane conductance regularor - inherited mutated gene
- protein that controls sodium and chloride channels
- disrupts the function of the chloride channel, preventing the usual flow of
chloride ions into and out of the channel
how does CF affect protein? causes it to malfunction or not be made at all
cystic fibrosis genetic mutation autosomal recessive
3 multiple choice options
what is the ratio of the inherit defective gene from both 1:4
parents for CF?
what is the likely hood of a child having CF if their 25%
parents are both carrier?
what is th elikely hood of a child being a carrier to CF if 50%
their parents are both carrier?
which organ systems are involved with CF? respiratory
GI
reproductive
integumetary
GI clinical manifestations due to CF meconium ileus
bowel obstructions
pancreatic enzyme deficiency
failure to thrive-poor growth
increased weight loss
diabetes risk
respiratory clinical manifestations due to CF risk for infections and gradual respiratory deterioration
COPD associated with infection
integumentary clinical manifestations due to CF sweat gland dysfunction
increase of sodium and chloride of sweat content
reproductive clinical manifestations due to CF infertility issues
diagnosis evaluation of CF abdominal x-ray to identify meconium ileus
chest x-ray to identify atelctasis & emphysema
pulmonary function tests
stool fat/enzyme analysis
72 hour stool sample with documented food intake
what is the most reliable diagnostic procedure for CF? sweat chloride test
normal levels for sweat chloride test chloride < 40mEql
sodium < 70mEql
diagnostic confirmation of sweat chloride test levels in chloride > 40mEql
infants under 3 months
diagnostic confirmation in all other ages chloride > 60 mEql