Renal function in pediatric cystic fibrosis patients in the first decade of life
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 m2, 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 m2) 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.
Keywords
Pediatrics Glomerular filtration rate Chronic kidney disease HyperfiltrationNotes
Acknowledgements
We would like to thank Ruth Milner for her statistical expertise and guidance, Dr. Amy Piepsz for the kind assistance and discussion around the issues of normal pediatric GFR values, and Dr. Douglas Matsell for his critical review of this paper in draft form. Finally, we would like to thank the Nuclear Medicine Department and CF clinic staff at BCCH who provide ongoing excellence of care for these patients.
Support and Financial Disclosure Declaration
No funding source was required for the preparation of this work. The authors have no financial or ethical conflicts of interest related to this manuscript.
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