Clinical Presentations Flashcards
Describe how a faulty CFTR protein causes problems with mucus
Chloride ions can’t move out of the cell into the ECF
Mucus becomes too viscous as water does not leave the cell in sufficient quantities
Too thick for cilia to move
Blocks ducts
How can CF cause problems in the GI tract?
Newborn effects
Adult effects
Causes mucus to become too viscous
Newborn: meconium ileus, inability to pass the first faeces as the mucus is not sufficiently hydrated
Older CF sufferers: constipation, invagination of the GI tract
How does absence of CFTR cause changes to sweat composition?
No CFTR in the apical membrane of epithelial cells lining the duct of the sweat gland
Results in poor reabsorption of chloride ions
As a result sweat is rich in sodium and chloride ions
Sweat tests for salty sweat to confirm CF
How does CF cause problems in the respiratory tract?
Mucus too thick
Can’t be moved by cilia
Debris and pathogens remain in the resp.tract
Prone to pulmonary infections
Coughing up mucus, physio
Breathing problems
How does CF cause problems in the pancreas?
Secretions contain too little water and become thickened
Exocrine ducts blocked
Exocrine pancrease becomes inflamed by digestive enzymes - painful pancreatities, fibrotic
The duodenum receives insufficient pancreatic digestive enzyme and malabsorption results
Insufficient lipase production results in faecal excretion of undigested fat, usually as diarrhoea. STEATORRHOEA
