Renal function in pediatric cystic fibrosis patients in the first decade of life
Chanel Prestidge1, Mark A Chilvers, A George F Davidson
1Department of Pediatrics, Division of Nephrology, BC Children's Hospital, 4480 Oak Street, ACB K4-151, Vancouver, British Columbia, Canada.
Insights
Cystic fibrosis (CF) patients show higher than normal kidney filtration rates, not significant impairment, despite potential medication effects. This study measured glomerular filtration rate (GFR) in children with CF.
Area of Science:
- Nephrology
- Pediatric Pulmonology
- Pharmacology
Background:
- Increasing life expectancy in cystic fibrosis (CF) patients leads to higher rates of chronic kidney disease (CKD).
- Limited data exists on baseline or longitudinal glomerular filtration rate (GFR) in pediatric CF populations.
- Exogenous clearance markers for GFR assessment are underutilized in this group.
Purpose of the Study:
- To assess renal function in pediatric patients with cystic fibrosis (CF).
- To determine the prevalence of chronic kidney disease (CKD) and measure glomerular filtration rate (GFR) using exogenous markers.
- To investigate potential links between limited aminoglycoside exposure and renal function in CF.
Main Methods:
- Retrospective cross-sectional study of 63 pediatric patients (2-18 years) at a single CF center.
- GFR measured via plasma disappearance of Technetium-99 m diethylenetriaminepentaacetic acid (mGFR).
- Renal dysfunction defined as CKD stage II or below, abnormal urinary sediment, or renal imaging.
Main Results:
- Four out of 63 patients exhibited signs of renal dysfunction (one with decreased mGFR, three with microscopic hematuria).
- Mean mGFR was significantly elevated at 140 ± 24 ml/min/1.73 m(2), exceeding expected values for healthy children.
- No significant renal impairment was detected following limited aminoglycoside exposure in the first decade post-CF diagnosis.
Conclusions:
- The study did not find significant renal impairment in pediatric CF patients with limited aminoglycoside exposure.
- Glomerular hyperfiltration was documented in a notable proportion of the studied CF population.
- Further research is needed to understand the long-term renal implications of hyperfiltration in CF.
Abstract:
With increasing life expectancy and the need for lung transplantation in the cystic fibrosis (CF) population, there are increasing reports of chronic kidney disease (CKD). However, values for baseline or longitudinal glomerular filtration rate (GFR) as measured by exogenous clearance markers are lacking in this population. Retrospective cross-sectional study in 2 to 18-year-olds cared for at a single CF center who had a GFR measured by plasma disappearance of Technetium-99 m diethylenetriaminepentaacetic acid (mGFR). The primary outcome was evidence of renal dysfunction as defined by CKD stage II or below (mGFR <90 ml/min/1.73 m(2), persistent abnormalities in urinary sediment, abnormal renal imaging). Of 63 patients evaluated, four had apparent renal dysfunction, one demonstrated decreased mGFR, and three others had persistent microscopic hematuria. The mean mGFR was substantially higher (140 ± 24 ml/min/1.73 m(2)) than expected or previously reported for healthy children. We did not demonstrate the presence of significant renal impairment after limited aminoglycoside exposure in the first decade following diagnosis with CF. However, we did document the presence of glomerular hyperfiltration in a significant proportion of our CF patients.
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