Cystic Fibrosis Flashcards
What gene is effected in CF?
CFTR gene
What is the name of the ion channel effected in CF?
Cystic fibrosis transmembrane regulator
In cystic fibrosis sufferers, what is different about the CFTR?
The ion channel is not present in the apical membranes of the epithelial cells
What does not happen in CF as a result of the lack of the CFTR?
Chloride ions are not transported across the membranes enough/at all
Why does someone with CF has such thick mucus?
Water does not the epithelium in sufficient quantities to adequately hydrate the secreted mucus. This is because there is no movement of chloride ions
What effect can viscous mucus have?
It is not readily moved to the oropharynx for swallowing. Meaning trapped pathogens and bacteria are able to thrive in the warm environment. Infections and illnesses are very likely
Why might the sweat of someone with CF be salty?
Because the CFTR is not there reabsorbing the chloride ions
What does having viscous mucous in the GI tract mean?
Newborns-meconium ileus (inability to pass first faeces)
Older- constipated and invagination of parts of the GI tract
What can viscous mucus lead to in the respiratory tract?
Serious pulmonary infection
What does CF cause in the exocrine parts of the pancreas?
Blocked ducts because the secretions contain too little water and are therefore thickened.
What can blocked ducts in the pancreas cause?
Pancreatitis and fibrotic
The gut receives insufficient pancreatic digestive enzymes and malabsorption occurs
The endocrine pancreas is also adversely affected
Why might a man with CF be infertile?
Because another feature of CF is the lack of vas deferens
What does insufficient lipase production result in for CF sufferers?
Faecal excretion of undigested fat, usually as diarrhoea
