Cystic Fibrosis Flashcards
is an inherited disease of the secretory glands
Cystic Fibrosis (CF)
The cystic Fibrosis disease is characterized by
thick mucus secretions, particularly in the pancreas and lungs, as well as electrolyte abnormalities in sweat gland secretions.
Cystic Fibrosis is inherited from an
autosomal recessive trait
______________ results in the inability to transport small molecules across cell membranes, leading to dehydration of epithelial cells
Abnormality of the long arm of chromosome 7
For early detection during pregnancy, ________ or _________ can be performed.
Chorionic Villi Sampling or Amniocentesis
Females affected by Cystic Fibrosis may have
thick cervical secretions that limit sperm motility
Affected males may be unable to reproduce secondary to persistent plugging and blocking of the vas deferens by
Tenacious seminal fluid
For cystic Fibrosis situation, patients who desire to become pregnant may have options like
*Alternative insemination
*In Vitro fertilization
the Acinar cells of the pancreas normally produce
*lipase
*trypsin
*amylase
Absence of pancreatic enzymes in the duodenum results in
inability to digest fat, protein, and some sugars.
Bowel movements become large, bulky, and greasy
Steatorrhea
An increase in the intestinal flora and fat results in
an extremely foul odor
increased bulk of feces often leads to
protuberant abdomen
Fat-soluble vitamins, particularly these vitamins cannot be absorbed in the absence of fat absorption resulting in vitamin deficiency
*Vitamin A
*Vitamin D
*Vitamin E
Meconium in newborn is normally
thick and tenacious
organisms most frequently cultured in CF situations
*Staphylococcus aureus
*Pseudomonas aeruginosa
*Haemophilus Influenzae
__________ may occur as a result of absorption of air from alveoli behind blocked bronchioles.
Atelectasis
The level of chloride to sodium in perspiration is increased
two to five time above normal
The level of chloride to sodium in perspiration is increased two to five time above normal resulting in
“salty” perspiration