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The Kidney and Cystic Fibrosis

Dilip Nazareth, MRCP, and Martin Walshaw, MD, FRCP

Cystic fibrosis (CF) is the most common inherited potentially fatal disease in the Western World, with an abnormal gene frequency of 4% and an incidence of approximately 1:2500 live births in the white population. Although the abnormality occurs in an epithelial cell ion transport protein, the CF conductance transmembrane regulator (CFTR), which is present in all mucus-producing organs, attention has previously focused on those systems in which the earliest damage occurs, specifically the lower airways and the digestive exocrine organs. However, with improvement in the management of CF individuals throughout their childhood years, nearly all now survive into adulthood. With increasing survival rates comes a raft of new complications, either as previously unexpected manifestations of the CF condition or as a consequence of a lifetime of necessarily aggressive treatment regimens. This review will look at the influence on the kidney of the CF gene product, not only in terms of its pathophysiology but also on the emerging and significant side-effects of potentially nephrotoxic therapy.

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