Abstract
Cystic Fibrosis (CF) is a unique disease in which the serum concentrations of many drugs are inexplicably lower than in subjects without CF. These lower concentrations are attributed to greater clearance of drugs from the body and larger apparent volumes of distribution, but why CF patients should exhibit these altered pharmacokinetic parameters remains an enigma. Examination of the major organs associated with drug disposition, specifically, the gastrointestinal tract, the heart, the liver, and the kidney, in patients with cystic fibrosis reveals some degree of functional impairment in each of these organs (1). In other diseases, impaired function of these organs results in higher concentrations and decreased clearances of drugs— the opposite of that which is observed in patients with CF. Therefore, CF presents an enigma both to the clinician who needs to treat patients with large numbers of drugs and to the scientist who must try to understand the disease and the reasons for altered drug disposition. However, great strides in CF research have been in the last few years and increasing interest is being focused on the use of drugs as probes to increase our understanding of the various components of abnormal tissue and organ function in CF (2).
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Spino, M. (1990). Pharmacokinetics of Drugs in Cystic Fibrosis. In: Moss, R.B. (eds) Cystic Fibrosis. Allergy and Immunology, vol 1. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-4612-0475-6_11
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