Renal impairment in children with cystic fibrosis

J Cyst Fibros. 2010 Jul;9(4):263-8. doi: 10.1016/j.jcf.2010.03.006. Epub 2010 Apr 21.


Background: Due to the improvement in life expectancy in cystic fibrosis (CF), co-morbidities such as renal function impairment may be more frequent.

Aim: To determine the prevalence of renal disease in children with CF and to identify associated risk factors.

Methods: A single-center retrospective study analyzing the genetic, clinical and therapeutic characteristics of 112 children. The estimated glomerular filtration rate (GFR), microalbuminuria and lithiasic risk factors were assessed.

Results: The median calculated GFR (Schwartz) was 123, 161 and 155ml/min/1.73m(2) in children aged 1, 6 and 15years, respectively. The cumulative dose of aminoglycosides was not correlated to GFR. Microalbuminuria was present in 22/38 patients. Hyperoxaluria was observed in 58/83 patients and was associated with a severe genotype, pancreas insufficiency and liver disease. Hypercalciuria, hyperuricuria and hypocitraturia were identified in 16/87, 15/83 and 57/76 patients, respectively.

Conclusion: Renal impairment in CF has various presentations. There appears to be low levels of renal impairment in children with CF. However, the risk of oxalocalcic urolithiasis is enhanced, and GFR may be underestimated by the Schwartz formula. Further studies using measured GFR techniques are thus warranted.

MeSH terms

  • Adolescent
  • Albuminuria / epidemiology
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications*
  • Female
  • France / epidemiology
  • Glomerular Filtration Rate
  • Humans
  • Hyperoxaluria / epidemiology*
  • Hyperoxaluria / etiology
  • Infant
  • Infant, Newborn
  • Longitudinal Studies
  • Male
  • Odds Ratio
  • Prevalence
  • Renal Insufficiency / epidemiology*
  • Renal Insufficiency / etiology*
  • Retrospective Studies
  • Urolithiasis / epidemiology*
  • Urolithiasis / etiology