MRCP UK EXAMINATIONS

Detailed explanation-1: -Cystic fibrosis (CF) is a disease that is passed down through families. It is caused by a defective gene that makes the body produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas.

Detailed explanation-2: -CF causes thick mucus to build up and clog certain parts of the body such as the lung. The buildup is caused by an abnormal gene called CFTR (cystic fibrosis transmembrane regulator). CFTR controls the flow of water and salt in and out of the body’s cells. Changes cause mucus to become thickened and sticky.

Detailed explanation-3: -In people with cystic fibrosis, mucus is dehydrated, becoming so thick and sticky that the cilia are unable to propel mucus out of the lungs. As a result, the mucus clogs the airways. The largest airway is the trachea between the throat and the lungs.

Detailed explanation-4: -CF mucus is often described as more solid and having a “flake” form. In addition, it has a higher concentration of proteins called mucins. Mucins make up the main component of mucus and are responsible for the gel-consistency of mucus. Non-CF mucus is composed mainly by two types of mucins: MUC5AC and MC5B.

Detailed explanation-5: -Mucus thinners (such as mucolytics) help thin and then move the mucus out of the airways so it can be coughed out. These medications can be taken through a nebulizer during ACTs. Inhaled antibiotics should be taken after ACTs are finished and the lungs are as clear of mucus as possible.