Journal of Cystic Fibrosis
Volume 9, Issue 4 , Pages 263-268, July 2010

Renal impairment in children with cystic fibrosis☆

  • Annick Andrieux

      Affiliations

    • CHU de Bordeaux, Centre de Ressources et de Compétences de la Mucoviscidose pédiatrique, Hôpital Pellegrin Enfants, Bordeaux, France
    • ,
  • Jérôme Harambat

      Affiliations

    • CHU de Bordeaux, Centre de Référence Maladies Rénales Rares (SORARE), Hôpital Pellegrin, Bordeaux, France
    • ,
  • Stéphanie Bui

      Affiliations

    • CHU de Bordeaux, Centre de Ressources et de Compétences de la Mucoviscidose pédiatrique, Hôpital Pellegrin Enfants, Bordeaux, France
    • ,
  • Fabienne Nacka

      Affiliations

    • Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'Investigation Clinique plurithématique, CHU de Bordeaux, France
    • ,
  • Albert Iron

      Affiliations

    • CHU de Bordeaux, Centre de Ressources et de Compétences de la Mucoviscidose pédiatrique, Hôpital Pellegrin Enfants, Bordeaux, France
    • ,
  • Brigitte Llanas

      Affiliations

    • CHU de Bordeaux, Centre de Référence Maladies Rénales Rares (SORARE), Hôpital Pellegrin, Bordeaux, France
    • ,
  • Michael Fayon

      Affiliations

    • CHU de Bordeaux, Centre de Ressources et de Compétences de la Mucoviscidose pédiatrique, Hôpital Pellegrin Enfants, Bordeaux, France
    • Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'Investigation Clinique plurithématique, CHU de Bordeaux, France
    • Corresponding Author InformationCorresponding author. Service de Pédiatrie, Unité de Pneumologie Pédiatrique, Hôpital Pellegrin Enfants, Place Amélie Raba-Léon, 33076 Bordeaux, France. Tel.: +33 5 5679; fax: +33 5 56795658.

Received 20 October 2009; received in revised form 6 March 2010; accepted 11 March 2010.

Abstract 

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/1.73m2 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.

Keywords: Cystic fibrosis, Child, Nephrotoxicity, Kidney disease, Urolithiasis, Antibiotics

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☆ Part of the data reported here has been presented at the annual meeting of the 32nd European Cystic Fibrosis Conference, 10–13rd June 2009, Brest, France, and printed in abstract form (J Cyst Fibros 8: S111, 2009).

PII: S1569-1993(10)00033-0

doi:10.1016/j.jcf.2010.03.006

Journal of Cystic Fibrosis
Volume 9, Issue 4 , Pages 263-268, July 2010

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