Renal impairment in children with cystic fibrosis

Annick Andrieux1, Jérôme Harambat, Stéphanie Bui

  • 1CHU de Bordeaux, Centre de Ressources et de Compétences de la Mucoviscidose pédiatrique, Hôpital Pellegrin Enfants, Bordeaux, France.

Insights

Children with cystic fibrosis (CF) show low renal impairment but an increased risk of kidney stones. Further research is needed to accurately assess kidney function in CF patients.

Area of Science:

  • Pediatric Nephrology
  • Cystic Fibrosis Research
  • Renal Function Assessment

Background:

  • Increasing life expectancy in cystic fibrosis (CF) leads to a higher incidence of comorbidities, including renal impairment.
  • Renal function monitoring is crucial in pediatric CF patients due to potential long-term complications.

Purpose of the Study:

  • To investigate the prevalence of renal disease in children with cystic fibrosis.
  • To identify risk factors associated with renal impairment in this population.

Main Methods:

  • A retrospective analysis of 112 pediatric CF patients was conducted.
  • Evaluated were estimated glomerular filtration rate (GFR), microalbuminuria, and lithiasic risk factors.
  • Genetic, clinical, and therapeutic data were collected and analyzed.

Main Results:

  • Median GFR (Schwartz) remained relatively stable across age groups (1-15 years).
  • Microalbuminuria was detected in 22/38 patients; hyperoxaluria in 58/83, linked to severe genotype, pancreatic insufficiency, and liver disease.
  • Abnormalities in hypercalciuria, hyperuricuria, and hypocitraturia were also observed.

Conclusions:

  • Renal impairment in CF presents diversely, with generally low levels detected in pediatric patients.
  • A significant risk for oxalocalcic urolithiasis exists in CF children.
  • The Schwartz formula may underestimate GFR; further studies with measured GFR are recommended.
Abstract

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