The association of nephrolithiasis with cystic fibrosis

Am J Kidney Dis. 2003 Jul;42(1):1-11. doi: 10.1016/s0272-6386(03)00403-7.


Background: There is a growing body of evidence regarding the association between cystic fibrosis (CF) and nephrolithiasis and the role that Oxalobacter formigenes may have in that association.

Methods: We performed a MEDLINE search of "cystic fibrosis and nephrolithiasis" and "Oxalobacter formigenes." Epidemiological and experimental evidence and possible mechanisms explaining the association were critically reviewed.

Results: Of patients with CF, 3.0% to 6.3% are affected with nephrolithiasis, a percentage greater than that of age-matched controls without CF, in whom the rate is 1% to 2%. Studies have suggested possible mechanisms for the association, including hyperuricosuria, hyperoxaluria, primary defects in calcium handling caused by mutation of the CF transmembrane regulator (CFTR), hypocitraturia, and lack of colonization with O formigenes, an enteric oxalate-degrading bacterium. The absence of colonization could be related to frequent courses of antibiotics.

Conclusion: Although the incidence of stones in patients with CF may be increased compared with controls without CF, many possible mechanisms are implicated. The relative contributions of these mechanisms remain uncertain. Future directions may include specific identification of lithogenic risks and therapy aimed at stone prevention in this population.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Animals
  • Calcium / metabolism
  • Calcium Oxalate / urine
  • Child
  • Chloride Channels / metabolism
  • Citrates / urine
  • Colon / microbiology
  • Crystallization
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / urine
  • Humans
  • Kidney Calculi / epidemiology
  • Kidney Calculi / etiology*
  • Malabsorption Syndromes / complications
  • Malabsorption Syndromes / metabolism
  • Oxalobacter formigenes / metabolism
  • Pancreatic Extracts / adverse effects
  • Prevalence
  • Rats
  • Uric Acid / urine


  • Chloride Channels
  • Citrates
  • Pancreatic Extracts
  • Calcium Oxalate
  • Uric Acid
  • Calcium