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Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 J+Pediatr 2016 ; 171 (ä): 196-201.e1 Nephropedia Template TP
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Kidney Disease Progression in Autosomal Recessive Polycystic Kidney Disease #MMPMID26831744
Dell KM; Matheson M; Hartung EA; Warady BA; Furth SL
J Pediatr 2016[Apr]; 171 (ä): 196-201.e1 PMID26831744show ga
Objective: To define glomerular filtration rate (GFR) decline, hypertension (HTN) and proteinuria in subjects with autosomal recessive polycystic kidney disease (ARPKD) and compare with two congenital kidney disease control groups in the Chronic Kidney Disease in Children (CKiD) cohort. Study design: GFR decline (iohexol clearance), rates of HTN (ambulatory/casual blood pressures (BPs)), antihypertensive medication usage, left ventricular hypertrophy (LVH) and proteinuria were analyzed in subjects with ARPKD (n=22) and two control groups: aplastic/hypoplastic/dysplastic (n=44) and obstructive uropathies (n=44). Differences between study groups were examined by Wilcoxon rank sum test. Results: Annualized GFR change in subjects with ARPKD was ?1.4 ml/min/1.73m2 (?6%), with higher decline in subjects age >10 years (?11.5%). However, overall rates of GFR decline did not differ significantly in subjects with ARPKD vs. controls. There were no significant differences in HTN or LVH rates, but subjects with ARPKD had a higher percent on ?3 BP medications (32% vs.0%, p<0.0001), more ACE inhibitor use (82% vs. 27% vs. 36%, p<0.0005), and less proteinuria (urine protein: creatinine=0.1 vs.0.6, p<0.005). Conclusions: This study reports rates of GFR decline, HTN and proteinuria in a small but well-phenotyped ARPKD cohort. The relatively slow rate of GFR decline in subjects with ARPKD and absence of significant proteinuria suggest that these standard clinical measures may have limited utility in assessing therapeutic interventions and highlight the need for other ARPKD kidney disease progression biomarkers.