Journal
AMERICAN JOURNAL OF NEPHROLOGY
Volume 44, Issue 3, Pages 171-178Publisher
KARGER
DOI: 10.1159/000448695
Keywords
Autosomal dominant polycystic kidney disease; Children; Epidemiology; Glomerular filtration rate; Hypertension; Longitudinal; Pediatrics; Total kidney volume
Categories
Funding
- National Institutes of Health [DK103678, DK097081, DK058793, DK034039, UL1 TR001082]
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Background: Long-term clinical outcomes in children with very-early onset (VEO; diagnosis in utero or within the first 18 months of life) autosomal dominant polycystic kidney disease (ADPKD) are currently not well understood. We conducted a longitudinal retrospective cohort study to assess the association between VEO status and adverse clinical outcomes. Methods: Seventy patients with VEO-ADPKD matched (by year of birth, sex and race/ethnicity) to 70 patients with non-VEO-ADPKD who participated in research at the University of Colorado were studied. Kaplan-Meier survival analysis was performed. The predictor was VEO status, and outcomes were progression to end-stage renal disease (ESRD), development of hypertension, progression to estimated glomerular filtration rate (eGFR <90 ml/min/1.73 m(2)), glomerular hyperfiltration (eGFR >= 140 ml/min/1.73 m(2)) and height-adjusted total kidney volume (htTKV) measured by MRI >= 600 ml/m. Results: Median follow-up was until 16.0 years of age. There were only 4 ESRD events during the follow-up period, all in the VEO group (p < 0.05). VEO patients were more likely to develop hypertension (hazard ratio, HR 3.15, 95% Cl 1.86-5.34; p < 0.0001) and to progress to eGFR <90 ml/min/1.73 m(2) (HR 1.97, 95% CI 1.01-3.84; p < 0.05) than non-VEO patients. There was no difference between groups in the development of glomerular hyperfiltration (HR 0.89, 95% CI 0.56-1.42; p = 0.62). There were only 7 patients who progressed to htTKV 6.00 ml/m, 4 in the VEO group and 3 in the non-VEO group (p < 0.01). Conclusions: Several clinical outcomes are worse in patients with VEO-ADPKD compared to non-VEO ADPKD. Children with VEO-ADPKD represent a particularly high-risk group of ADPKD patients. (C) 2016 S. Karger AG, Basel
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