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Cystic Fibrosis

The absence of phenylalanine from the transmembrane regulator that influences transport of sodium and water across cell membranes of duct or airway lining epithelium will give rise to the clinical disorder of cystic fibrosis. A minority has other genetic abnormalities that produce the same disorder.

The result in the airway, pancreatic and biliary system is to reduce the amount of water in the mucous secretions, making them more viscus. The reduction of salt and water reabsorption in the sweat glands will account for the high sodium in the sweat of these patients.

Respiratory complications are increased susceptibility to infection and bronchiectasis, which is not limited to dependent segments.

Early recognition, intensive physiotherapy, moisturising, bronchial irrigation with saline, bronchodilators, pancreatic supplements and prompt treatment of infections all contribute to increased survival so that increasingly, the greater proportion of living cystic fibrosis patients is adult.

The penalty for increased survival is the greater incidence of the complications from other ducts that are susceptible to the presence abnormal viscid mucus, such as small hepatic bile duct obstruction and stenosis. Pancreatic insufficiency is more common and affects 80 percent of children with cystic fibrosis, being the accumulation of small foci of pancreatitis leading to progressive loss of pancreatic substance and function. The islet cells are not spared and diabetes mellitus complicates the long standing pancreatitis.

Various anatomic expressions of pathology.

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[London South Bank U.]

IDM April 2006