Cystic fibrosis and renal disease

Paediatr Respir Rev. 2002 Jun;3(2):135-8.


Renal disease is relatively rare in cystic fibrosis even though the cystic fibrosis transmembrane regulator (CFTR) protein is expressed in abundance in the kidney. Aberrant CFTR expression probably explains the subtle abnormalities in renal concentrating and diluting ability described in cystic fibrosis and possibly the increased incidence of nephrocalcinosis. It also provides a hypothesis for the important differences in renal handling of some drugs. As the prognosis improves for patients with cystic fibrosis, secondary renal complications, for example glomerulonephritis and amyloidosis, are likely to become more prevalent. There are also a number of potentially nephrotoxic drugs used in the management of cystic fibrosis.

MeSH terms

  • Amyloidosis / complications
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Diabetic Nephropathies / complications
  • Glomerulonephritis, IGA / complications
  • Humans
  • Kidney Calculi / complications
  • Kidney Diseases / chemically induced
  • Kidney Diseases / complications*
  • Nephritis, Interstitial / complications
  • Nephrocalcinosis / complications


  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator