Cystic Fibrosis Flashcards
Why are mucus secretions vicious in CF sufferers and what are the consequences of this in the Respiratory Tract?
Chloride ion transport is compromised due to the lack of CFTR and so water does not leave epithelium and mucus does not stay hydrated. Viscous mucus does not move up to the oropharynx easily and so pulmonary infection can result.
What is Meconium Ileus?
Inability in a newborn to pass the first faeces. In older CF sufferers the equivalent is constipation and sometimes invagination of parts of the bowel.
Why is the sweat of CF sufferers salty?
The lack of reabsorption of chloride ions in the sweat glands means that sweat is high in both sodium and chloride ions and tastes salty.
What problems can occur in the pancreas of CF sufferers?
Exocrine secretions contain too little water and are too thick. Ducts become blocked causing the pancreas to become inflamed and fibrotic (Fibrotic disease of the pancreas).
Why do CF sufferers suffer from diarrhoea?
If the ducts in the pancreas become blocked then insufficient digestive enzyme enters the gut. This means that some substances are not fully digested (fats) and excreted in faeces. The lower water potential in the gut draws water from epithelial cells.
What is the problem in Cystic fibrosis?
Ion channel, cystic fibrosis transmembrane regulator (CFTR) is not present on the apical cell surface of epithelial cells in CF sufferers.
